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CME

Microtia Reconstruction
Gordon H. Wilkes, M.D.
Learning Objectives: After reviewing this article, the participant should be
Joshua Wong, M.D., M.Sc.
able to understand: 1. The epidemiology and genetics of microtia. 2. Refine-
Regan Guilfoyle, M.D.
ments in surgical technique for microtia. 3. Outcomes of treatment. 4. Chal-
Edmonton, Alberta, Canada lenges in treatment selection, hearing restoration, surgical training, and tissue
engineering.
Summary: Microtia reconstruction is both challenging and controversial. Our
understanding of the epidemiology and genetics of microtia is improving. Sur-
gical techniques continue to evolve, with better results. Treatment selection
continues to be controversial. There are strong proponents for reconstruction
with costal cartilage, Medpor or a prosthesis. More realistic models for teach-
ing surgeons how to do the procedures are becoming available. Our approach
to hearing rehabilitation is changing. Better solutions using percutaneous and
implantable devices are under evaluation to help both unilateral and bilateral
microtia patients. Tissue engineering will offer some exciting new treatment
possibilities in the future.  (Plast. Reconstr. Surg. 134: 464e, 2014.)

E
ar reconstruction continues to be chal- genetic and environmental contributions to
lenging and not without controversy. microtia. There are more than 18 different
There have been several CME and review microtia-associated syndromes with single-gene
articles published in this Journal and others, or chromosomal aberrations; however, there is
beginning with the excellent two-part series by no causal genetic mutation confirmed to date
Drs. Beahm and Walton in 2002.1–9 This series in (Table 1). Mendelian inheritance is more com-
particular gives an in-depth overview of the field mon in syndromic and familial cases. Multifac-
of ear reconstruction at the time, and much is torial or polygenic causes are more probable
still very relevant. When used with this over- in sporadic cases. Microtia and oculoauriculo-
view, together they provide an excellent under- vertebral spectrum share variable phenotypic
standing of the classic surgical techniques and expression, asymmetric facial involvement,
their evolution. The goal of this CME article right-side preponderance, male predilection,
is to attempt to minimize repetition and pres- familial occurrence, microtia, tags, and pits.
ent complementary new information. Most ref- Craniofacial or hemifacial microsomia and
erences have been published since 2005. This Goldenhar syndrome are included in this
article in conjunction with these other reviews spectrum. There are no accepted minimal
will give the reader an up-to-date understanding diagnostic criteria for oculoauriculovertebral
of the field of ear reconstruction and its areas of spectrum. Microtia and oculoauriculoverte-
controversy. bral spectrum should each be considered a
separate entity. Current hypotheses for micro-
tia include (1) neural crest cell disturbance,
EPIDEMIOLOGY AND GENETICS OF
(2) vascular disruption by means of several
MICROTIA
The cause and wide variation in prevalence
(0.83 to 17.4 per 10,000 births) is still poorly Disclosure: The authors have no financial interest
understood.10 There is evidence for significant to declare in relation to the content of this article.

From the Institute for Reconstructive Sciences in Medicine,


Covenant Health Group, Faculty of Medicine and Dentistry, Related Video content is available for this ar-
Misericordia Community Hospital. ticle. The videos can be found under the “Re-
Received for publication September 18, 2013; accepted May lated Videos” section of the full-text article, or,
28, 2014. for Ovid users, using the URL citations pub-
Copyright © 2014 by the American Society of Plastic Surgeons lished in the article.
DOI: 10.1097/PRS.0000000000000526

464e www.PRSJournal.com
Volume 134, Number 3 • Microtia Reconstruction

Table 1.  Human Disorders with Microtia (Except Chromosomopathies)*


Syndrome Microtia (%)† Genes Identified
Bixler (hypertelorism-microtia-clefting) 100 —
Bosley-Salih-Alorainy 33 HOXA1
Branchiooculofacial 20 TFAP2A
Branchiootic 80–90 EYA1, SIX1
Branchiootorenal 30–60 EYA1, SIX5
CHARGE Reported CHD7, SEMA3E
Congenital deafness, inner ear agenesis, microtia, and microdontia 100 FGF3‡
Craniofacial microsomia 65 —
Fraser Reported FRAS1, FREM2
Kabuki 80 MLL2
Klippel-Feil Reported GDF6
Lacrimoauriculodentodigital 20 FGFR2, FGFR3, FGF10
Mandibulofacial dysostosis 100 HOXD
Meier-Gorlin (ear-patella-short stature) 100 ORC1, ORC4, ORC6, CDT1, CDC6
Microtia, hearing impairment, and cleft palate 100 HOXA2
Miller 100 DHODH
Nager 80 —
Oculoauricular 100 HMX1
Pallister Hall Reported GLI3
Townes-Brocks 20 SALL1
Treacher Collins 60–80 TCOF1
Wildervanck (cervicooculoacoustic) Reported
*Reprinted with permission from Luquetti DV, Heike CL, Hing AV, Cunningham ML, Cox TC. Microtia: Epidemiology and genetics. Am J Med
Genet Part A 2012;158:124–139.
†Gorlin RJ, Cohen MM, Hennekam RCM. Syndromes of the Head and Neck. Oxford, NY: Oxford University Press; 2001.
‡Hemizygosity: only inner ear anomalies.

Table 2.  Risk Factors for Microtia Reported in the SURGICAL TECHNIQUE FOR MICROTIA
Literature*
First-Stage Modifications
General Surgical creation of a three-dimensional cos-
 Male sex
 Low birthweight tal cartilage ear framework has traditionally been
 First parity considered the most challenging part of ear
 High parity reconstruction.11–15 Classically, Brent harvested
 Multiple births
 Maternal acute illnesses contralateral rib cartilage in an extraperichon-
 Maternal type 1 diabetes drial plane. Nagata and Firmin both harvest carti-
 Maternal use of medications lage from the ipsilateral chest. Nagata harvests in
 Advanced paternal age
 Advanced maternal age a completely subperichondrial plane and Firmin
 Low maternal education leaves the posterior perichondrium intact. Inad-
 Maternal exposure to altitude vertent pleural tear and chest wall deformity are
 Maternal residence in an urban area
 Maternal residence in a rural area less likely if perichondrium is left intact. Some sur-
 Maternal exposure to air pollution geons feel there is better adherence of soft tissues
Race/ethnicity to the framework if some perichondrium is left
 Native ethnicity
 Hispanic ethnicity behind following carving; however, Nagata has not
 Ecuadorian found this to be a problem. Modifications of the
 Chilean Brent and Nagata techniques particularly involv-
 Asian
 Philippine ing the tragal and conchal bowl regions continue
 Pacific Islander to be reported. Included are three videos of cos-
Teratogens
 Retinoic acid tal cartilage ear framework creation. (See Video,
 Thalidomide Supplemental Digital Content 1, which displays
 Alcohol the fabrication of the three-dimensional costal car-
 Mycophenolate mofetil
tilage frame for microtia. This video is available in
*Used with permission from Luquetti DV, Heike CL, Hing AV, Cun-
ningham ML, Cox TC. Microtia: Epidemiology and genetics. Am J the “Related Videos” section of the full-text article
Med Genet Part A 2012;158:124–139. on PRSJournal.com or, for Ovid users, at http://
links.lww.com/PRS/B55. See Video, Supplemen-
different mechanisms, and (3) altitude. There tal Digital Content 2, which demonstrates how
are a multitude of potential risk factors for the to carve an autologous rib cartilage. This video
development of microtia (Table 2). is available in the “Related Videos” section of the

465e
Plastic and Reconstructive Surgery • September 2014

Video 1. Supplemental Digital Content 1, which displays the fabrica-


tion of the three-dimensional costal cartilage frame for microtia, is avail-
able in the “Related Videos” section of the full-text article on PRSJournal.
com or, for Ovid users, at http://links.lww.com/PRS/B55. Courtesy of S.
Nagata, ©Nagata Microtia & Reconstructive Plastic Surgery Clinic.

Video 2. Supplemental Digital Content 2, which demonstrates how


to carve an autologous rib cartilage, is available in the “Related Vid-
eos” section of the full-text article on PRSJournal.com or, for Ovid
users, at http://links.lww.com/PRS/B56. Courtesy of F. Firmin.

Video 3. Supplemental Digital Content 3, which shows the first


stage in total auricular reconstruction, is available in the “Related
Videos” section of the full-text article on PRSJournal.com or, for Ovid
users, at http://links.lww.com/PRS/B57. Courtesy of R. Zhang.

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Volume 134, Number 3 • Microtia Reconstruction

full-text article on PRSJournal.com or, for Ovid Creation of a Postauricular Sulcus


users, at http://links.lww.com/PRS/B56. See Video, Successful elevation of the ear framework and
Supplemental Digital Content 3, which shows the creation of a postauricular sulcus continues to be
first stage in total auricular reconstruction. This a challenge. Past approaches have included ele-
video is available in the “Related Videos” section vation and placement of a split-thickness or full-
of the full-text article on PRSJournal.com or, for thickness graft only. Addition of a surgical stent
to maintain projection has been used. The results
Ovid users, at http://links.lww.com/PRS/B57.)
were variable and often poor. New refinements
Nagata adds an extra piece of cartilage under appear to be beneficial, including adding a car-
the framework to deepen the conchal bowl. Firmin tilage, bone, or alloplastic buttress.20–22 Alloplastic
adds an extra piece she calls the “surelevation” to materials include bone cement and a hydroxyapa-
give more stability and projection to the root of tite ceramic. During elevation, Brent and Nagata
the helix and tragus. Chin et al.16 place an “extra leave some soft tissues on the elevated cartilage.
cartilaginous cube” under the reconstructed tra- Firmin exposes the entire posterior surface of the
gus to give better projection and stability. They framework during elevation, leaving no soft tis-
add a piece of cartilage to reconstruct the anti- sues restraining the projection except for the pos-
helix only if the thickness of the cartilage block is terior wall of the concha. The buttress requires
vascularized coverage. Flaps described include a
less than 5 mm. More recently, however, greater
postauricular fascial flap, a temporoparietal fas-
emphasis has been given to soft-tissue manage- cial flap, and a superficial musculoaponeurotic
ment to optimize the aesthetic result. Both Firmin system (SMAS) advancement flap. The flap from
and Marchac17 and Park16 provide an algorithm to the mastoid is thicker than a temporoparietal fas-
help with the soft-tissue management of microtia. cial flap and most commonly covers just the but-
It has been shown that the subcutaneous pedicle tress. The temporoparietal fascial flap can cover
in Nagata’s W flaps does increase the vascularity the complete raw surface of the posterior ear but
and decrease skin flap necrosis18,19 (Fig. 1). A gap is more involved to elevate. Endoscopic harvest
is needed between the tip of the tragus and crus has been reported, with fewer alopecia issues.23
helicis in the carved framework to allow rotation Three different techniques of sulcus construc-
tion in microtia repair using a temporoparietal
around the pedicle during framework placement
fascial flap, a retroauricular fascial flap from the
if the pedicle is left intact. mastoid region, and an SMAS advancement flap
were compared, with no significant difference in
outcome at 3 months. All included a cartilage but-
tress.24 The authors found the SMAS flap safe and
easy to perform, and the procedure resulted in no
secondary defects. It can be used for combined
auricle and middle ear reconstructions, leaving
the temporoparietal fascial flap available for com-
plicated or revision cases. Rotation and advance-
ment flaps have been described.25 The technique
described by Chen et al. involves elevating a thin
split-thickness skin graft from hair-bearing scalp
in continuity with the full-thickness skin posterior
to the ear to cover the temporoparietal fascial flap
and avoid some visible scarring.26 Primary graft
healing is essential in all techniques to prevent sec-
ondary wound contraction and loss of projection.

Harvesting the Rib and Chest Wall Deformity


Critics of autogenous reconstruction cite chest
wall deformity as a major drawback. Although still
Fig. 1. Lobule-type microtia marked for Nagata-type reconstruc- an issue, it appears to be less of a problem. Cer-
tion. Circle indicates where the subcutaneous pedicle of tissue is tainly, rib harvest including the perichondrium
left for circulation to the W-type flaps. can result in chest wall deformity.27,28 Brent has

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Plastic and Reconstructive Surgery • September 2014

advocated leaving a rim of sixth costal cartilage contralateral free vascularized temporoparietal fas-
in situ to mitigate against this. Further steps are cial flaps. The potential for complications including
being taken intraoperatively to lessen the possible skin necrosis and the added surgical stage are the
adverse effect of harvesting rib cartilage.29 Fir- major concerns with the use of soft-tissue expanders
min recommends leaving the posterior perichon- in ear reconstruction.35-37 Many complications are
drium intact, stating that the “deformation at the preventable with proper planning (Table 3). Higher
donor site is minimal.” Kawanabe and Nagata30,31 rates of skin necrosis in acquired versus congenital
leave the complete perichondrial sleeve intact and cases have been reported. Expanding scarred skin
fill it or a Vicryl (Ethicon, Inc., Somerville, N.J.) provides inadequate skin in both quality and quan-
sleeve with diced pieces of leftover cartilage. In a tity for the delicate needs of ear reconstruction and
series of 273 patients, they reported no chest wall should be discouraged.
deformity. They also demonstrated regeneration
of cartilage for future use. Siegert and Magritz32
described reducing chest wall morbidity by using OUTCOMES
patient-controlled analgesia for pain and a two- There is no commonly accepted means of
stage approach to hide the scar in the inframam- assessing outcomes in ear reconstruction. Per-
mary fold and to provide a full-thickness skin graft ceived results as judged by the patient, family,
in women, and minimizing chest wall deformity surgeon, and other observer can be quite differ-
by leaving perichondrium intact posteriorly and ent. The psychological outcome of treatment is as
placing cartilage pieces in an absorbable sleeve to important as the patient’s overall recovery.
make “new” regenerated ribs. Microtia patients have been found to have a
high prevalence of mood disorders, in particular,
Soft-Tissue Expanders depression, interpersonal/social difficulties, and
The use of soft-tissue expanders in ear recon- hostility/aggression.38 The child usually discovers
struction can be helpful.33,34 The rationale is to they are different around the age of 3 or 4 years.
attempt to provide complete skin coverage of a The age of surgical intervention must be balanced
framework with no need for a fascial flap or skin against the potential for a good aesthetic reconstruc-
graft. This approach provides thin skin with good tion, as a poor result may have a long-term adverse
color match. Barring a complication during expan- psychological effect on self-esteem. The earlier the
sion, the circulation is reasonable, as the skin child is made aware of the deformation, the lower
has been delayed. Flaps can be designed in the the prevalence of psychological disorders.
expanded skin to provide coverage both anteriorly A standardized assessment tool (Glasgow Ben-
and in the posterior sulcus. Dealing with the cap- efit Inventory) measuring health-related quality
sule around the soft-tissue expander is controver- of life and a surgical outcome questionnaire have
sial. It can be removed judiciously in areas if it is too been effectively applied to ear reconstruction.
thick or would affect adherence to the underlying Soukup et al. showed a significant improvement
structures. It is safest to leave it attached to any flaps in health-related quality of life following autog-
created in the expanded skin. Soft-tissue expanders enous ear reconstruction (Level of Evidence:
have also been used to expand under a temporo- ­Therapeutic, IV).39 The Glasgow Benefit Inven-
parietal fascial flap and scalp skin graft to provide tory demonstrated the greatest impact helping
coverage in challenging cases of anotia, failed autog- patients improve social interaction and relation-
enous reconstructions, and posttraumatic cases. ships. Microtia with a syndrome had higher scores
This has included both ipsilateral pedicled and than isolated microtia. Age at the time of surgery
had no effect. Chest scar color was more concern-
ing than chest wall deformity. All had some form of
Table 3.  Causes of Skin Necrosis Using Soft-Tissue successful chest wall reconstruction. Surprisingly,
Expanders in Ear Reconstruction* the surgeons and age-matched patients gave lower
Wrong expander choice scores than the patients and parents. This contra-
Valve placed over bony prominence dicts opinions held by many skeptics that surgeons
Fold in STE
Overinjection overestimate the quality of their results. Overall,
Scarring in the area there was a strong correlation between Glasgow
Poor incision placement Benefit Inventory and the surgical outcome
STE, soft-tissue expanders. scores. This study identifies where to improve the
*From Jing C, Hong-Xing Z. Partial necrosis of expanding postau-
ricular flaps during auricle reconstruction: Risk factors and effective most to achieve greater health-related quality-of-
management. Plast Reconstr Surg. 2007;119:1759–1766. life benefits. Others have shown similar results

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Volume 134, Number 3 • Microtia Reconstruction

in both retrospective and prospective studies.40–42 implant stability are minimal. The major disad-
Approximately 70 percent of patients felt the ear vantages of the osseointegrated approach are
became part of the body image and the chest scar intermittent, usually mild, chronic soft-tissue
was acceptable. Interestingly, they revealed no dif- problems; the need for continued maintenance;
ference in lifestyle and self-consciousness between and repeated prosthetic reconstruction every 2
autogenous and prosthetic treatment patients. to 5 years. The implant failure rate in nonirradi-
Braun et al. used the Glasgow Benefit Inven- ated mastoid bone is low (2 percent), and loss of
tory, the Glasgow Children’s Benefit Inventory, an implant does not necessarily mean inability to
and a questionnaire to study a group of patients wear the prosthesis. The majority of patients (97
who underwent reconstruction with porous poly- percent) were satisfied. Despite the soft-tissue
ethylene (Medpor; Porex Surgical, Inc., Newnan, problems, 94 percent would do it again and 97
Ga.) (Level of Evidence: Therapeutic, IV).43 There percent would recommend it to other patients.
was again a high satisfaction rate with the aesthet-
ics in 73 percent of adults and 85 percent of chil-
dren. The Glasgow Benefit Inventory was elevated TREATMENT SELECTION
(21.2), indicating improvement in health-related Controversies remain regarding appropriate
quality of life, but was not as high as in the autog- treatment selection for patients with major ear
enous group (48.1). deformities. These include choice of framework
The results of various autogenous techniques (either costal cartilage or Medpor), surgical tech-
have recently been published by several authors.44–46 nique, or type of reconstruction (osseointegrated
Although overall satisfaction is reported as high, prosthetic or autogenous). Although we all have
there are certainly weaknesses in the methods of treatment biases, providing informed consent
evaluation. When performed properly in an appro- requires up-to-date knowledge of the various
priate patient by an experienced surgeon, a satis- approaches and their appropriateness in a variety
factory, stable ear reconstruction is possible in the of clinical situations (Table 4).
majority of cases. Probably more important than
the specific technique is the overall experience of Autogenous
the surgeon using an acceptable technique. Critics Although most surgeons would agree that a
of autogenous reconstruction argue that the results successful autogenous ear reconstruction is ideal,
published by the leaders in the field are only the critics would argue that currently the aesthetic
best results and are not necessarily obtainable by results are very inconsistent and often poor. Con-
other surgeons performing smaller volumes. tinued refinements in surgical techniques have
Controversy still surrounds the outcomes of a
resulted in better ear reconstructions (Fig. 2).
porous polyethylene alloplastic framework. Rein-
The results are becoming more consistent and
isch and Lewin published their series of 786 ear
reconstructions from 1991 to 2008.47 Complica-
tions decreased when complete coverage of the Table 4.  Indications for Specific Techniques in Ear
framework with a temporoparietal fascial flap was Reconstruction
used. A subgroup of 41 temporoparietal fascial flap
patients with 12 years’ follow-up had a 2.7 percent Autogenous
 Microtia
fracture rate and a 7.3 percent exposure rate. Braun  Lower third intact
et al. presented their 65 temporoparietal fascial flap  Patient preference
patients with a porous polyethylene framework.48  Less compliant patient
 In compromised area if TPFF available
Twenty-eight patients (43.1 percent) had one or Osseoprosthetic
more revision operations, mainly for minor correc-  Severe local trauma and scarring
tions. Only one required a major revision opera-  Thermal injury
 After oncologic resection
tion with partial explantation and reimplantation  After radiotherapy
of porous polyethylene. Others have also reported  Poor autogenous result
success with porous polyethylene frameworks.49,50  Patient preference
 Poor operative risk
Osseointegrated prosthetic ear reconstruc- PPE framework
tion has also demonstrated high levels of success  Patient preference
and patient satisfaction.51,52 Psychologically, the  Calcified costal cartilage
 Patient does not want rib harvested
prosthesis becomes part of the body image in the  Microtia (controversial)
majority of patients. Issues regarding prosthetic  In compromised area if TPFF available
shape, color, means of attachment, and long-term TPFF, temporoparietal fascial flap; PPE, porous polyethylene.

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Plastic and Reconstructive Surgery • September 2014

ear. Proponents tout several advantages.47,49,50 A


less controversial indication for the use of a porous
polyethylene framework is the well-informed adult
who does not want costal cartilage harvested or in
whom it is too calcified and who does not want a
prosthesis.

Osseointegrated Auricular Prosthetic


Reconstruction
Many plastic surgeons have difficulty under-
standing the role of this modality. Osseointe-
grated auricular prosthetic reconstruction is

Fig. 2. Successful reconstruction of lobule type microtia using a


Nagata-type technique.

Table 5.  Advantages of Specific Techniques in Ear


Reconstruction
Autogenous
 Uses your own tissues
 Long-term stability
 No ongoing care
 Can use in less compliant patient Fig. 3. Major soft-tissue injury including traumatic ear loss fol-
Osseoprosthetic lowing a motor vehicle accident.
 Outpatient, straightforward surgery
 Useful in elderly or medically compromised patients
(can perform under local anesthesia)
 Can use in severely compromised tissues
 Good for tumor surveillance
 Prosthesis very realistic
PPE framework
 No chest morbidity
 Microtia reconstruction at younger age
 Framework shape relatively consistent
PPE, porous polyethylene.

reproducible. Surgeons are more aware of the


need for appropriate training and mentorship
and a sufficient case volume to achieve consistent
high-quality reconstructions. Most reconstructions
currently performed are variations of the Brent or
Nagata technique. Although they involve more
complicated frameworks, Nagata-type procedures
are becoming much more common (Table 5).

Porous Polyethylene Framework (Medpor)


The role of porous polyethylene frameworks
in our treatment selection remains controversial.
There remains reluctance by many to use an allo- Fig. 4. The same patient following soft-tissue expansion of the
plastic framework in an exposed area such as the cheek and temple and osseointegrated ear prosthesis.

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Volume 134, Number 3 • Microtia Reconstruction

Table 6.  Disadvantages of Specific Techniques in Ear


Reconstruction
Autogenous
 Variability of aesthetic result
 More surgical stages
 Longer surgical procedures
 Chest wall morbidity
 Steep learning curve
Osseoprosthetic
 Intermittent soft-tissues problems
 Long-term maintenance
 Prosthetic remakes every 2–5 yr
 Ongoing cost
 Can compromise future autogenous options
 Need compliant patient
PPE framework
 Uses temporoparietal fascial flap
 Long-term risk of alloplastic implant exposure or loss
 Compromise any future autogenous options
PPE, porous polyethylene.

Table 7.  Patient Satisfaction after Different Types of


Ear Reconstruction
Fig. 5. Failed autogenous ear reconstruction after over 10 surgi-
cal procedures. Satisfaction Would You Do It
Rate (%) Again? (%)
Autogenous
 Zhang 89
 Brent 90
 Siegert 82 90
 Kristiansen 95
Osseointegrated prosthetic
 Korus et al. 97 94
 Younis et al. 60 70
Medpor
 Braun et al.
   Pediatric patients 85 90
   Adult patients 73 87

of microtia, it is our opinion that a reasonable


autogenous result is a superior long-term treat-
ment choice (Table 6).
The onus is on the reconstructive surgeon
to consistently provide a reasonable result with
minimal morbidity and a high satisfaction rate
(Table 7). As all these approaches can produce
Fig. 6. The same patient has successfully worn an osseointe- a satisfied patient, appropriate treatment selec-
grated ear prosthesis for 24 years. tion and informed consent are some of the more
important issues facing the surgeon in dealing
complementary to other approaches and pro- with patients with major ear deformities. It is our
vides a reasonable alternative in many cases with experience that when all the options are pre-
poor autogenous options (Figs. 3 and 4) or a sented, patients decide quickly how to proceed
poor autogenous result (Figs. 5 and 6). The use and very rarely change their mind.
of an adhesive-retained prosthesis should not be
considered a trial for an osseointegrated auricu-
lar prosthetic. Stability, positioning, skin break- HEARING RESTORATION IN THE
down, and confidence issues are completely MANAGEMENT OF MICROTIA
different when using adhesives. Although some The treatment of microtia should ideally involve
surgeons consider osseointegrated auricular reconstruction of the external ear and the resto-
prosthetic reconstruction a primary treatment ration of normal hearing. Hearing impairment

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Plastic and Reconstructive Surgery • September 2014

Table 8.  Deleterious Effects of Untreated Unilateral not depend on a functioning middle ear or a pat-
Hearing Loss* ent canal. The most common practice is placement
of a unilateral bone-anchored hearing aid in bilat-
Poorer language scores or delayed language development
Increased rates of grade failure (22–35%) eral microtia and bilateral conductive hearing loss
Increased need for educational assistance (12–41%) patients because a single hearing aid will stimulate
Increased behavioral issues in the classroom both cochleae simultaneously. Recent evidence
*From Lieu JE. Speech-language and educational consequences of suggests that restoration of binaural hearing in
unilateral hearing loss in children. Arch Otolaryngol Head Neck Surg.
2004;130:524–530; and Lieu JE, Tye-Murray N, Karzon RK, Piccirillo fact results in greater stimulation of the cochlea
JF. Unilateral hearing loss is associated with worse speech-language and better directional hearing, space perception,
scores in children. Pediatrics 2010;125:e1348–e1355. and speech recognition in noise.58–63 Janssen et al.
summarized these findings in a recent systematic
conceptually impairs a variety of social, cognitive, review of 11 articles, concluding that bilateral bone-
and developmental domains (Table 8). Hearing anchored hearing aid use results in improved hear-
impairment in microtia is related to abnormali- ing sensitivity and speech perception in the quiet,
ties of the external auditory canal, tympanic mem- speech perception in noise, localization and later-
brane, and middle ear. Attempts to reconstruct alization, and patient perception of quality of life
these structures have been fraught with difficul- and overall sound quality.64 With this evidence, the
ties such as frequent restenosis, infection, possible verdict is still controversial but warrants expand-
facial nerve injury, and scarring at the future site ing our therapeutic options to those with bilateral
for auricular reconstruction.53 Hearing improve- microtia and bilateral conductive hearing.
ment has been variable and often poor. For these Thoughts are evolving in unilateral microtia
reasons, traditional thinking has been that further patients with unilateral conductive hearing loss. Tra-
intervention is not necessary in unilateral microtia ditional thinking was that hearing on a single side
if there is normal hearing in the other ear. Yeakley was sufficient for speech development and hearing
and Jahrsdoerfer developed a computed tomo- in education. A recent review of auricular recon-
graphic grading system to predict patients with struction states that most children with unilateral
the most favorable hearing outcome from surgical microtia are born “adjusted to their monaural con-
intervention.54 Their results have proven difficult dition.”12 Studies suggest that the educational and
to replicate by most otologists. Also, comparative cognitive developmental consequences of unilat-
studies have shown that reconstructive middle ear eral hearing loss warrant reevaluation of traditional
surgery often requires the addition of an air-con- practice and application of early treatment (Ref-
duction hearing aid for hearing to be as good as erence 66 Level of Evidence: Therapeutic, IV).65–67
that of a bone-anchored hearing aid alone.55 Sieg- Evidence indicates that there is both audiologic
ert56 describes reconstruction of an ear canal and and subjective benefit when treating unilateral
tympanic membrane as part of a three-stage micro- conductive hearing with a bone-anchored hear-
tia reconstruction. At the first stage, remnants of ing aid.68–70 Further study is needed and treatment
elastic auricular cartilage are packed densely into must be individualized. Giving patients the option
silastic mold to prefabricate the tympanic mem- of trying a cutaneous bone conduction hearing
brane. The external ear canal is also prefabricated aid (i.e., Softband; Cochlear) in consideration of
with rib hyaline cartilage positioned in a silastic a permanent bone-anchored hearing aid is reason-
cylinder. These are stored in the subcutaneous tho- able.71 Newer implantable hearing devices such as
racic wound. At the second stage, the ear frame- the Vibrant Soundbridge and Bonebridge (Med-El
work is mobilized and the prefabricated tympanic Corp., Innsbruck, Austria) and the Sophono Sys-
membrane and external ear canal are placed. At tem (Sophono Inc., Boulder, Colorado) are now
the third stage, the canal is exteriorized and skin being studied. Their long-term success in hearing
grafted. In a later publication, he states there was restoration and effects on external ear reconstruc-
no restenosis of the canal but “reaching a near- tion still require further evaluation.72–75
normal hearing is not the rule.” There continues
to be further investigation of surgical options. Autogenous Ear Reconstruction and the
Bilateral microtia patients have unique chal- Bone-Anchored Hearing Aid
lenges for hearing restoration. The bone-anchored There are obvious conflicting needs between
hearing aid (BAHA; Cochlear, Mölnlycke, Sweden; the otologist and the reconstructive surgeon
and Ponto; Oticon, Kongeballen, Denmark) Cen- regarding placement of the bone-anchored hear-
tennial, Col.) has been used since 1977, relying on ing aid and the timing of surgery. Appropriate
bone conduction directly to the cochlea.57 It does placement of the bone-anchored hearing aid is

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Volume 134, Number 3 • Microtia Reconstruction

Fig. 7. Planning bone-anchored hearing aid implant site place- Fig. 9. Planning bone-anchored hearing aid implant site place-
ment using a “dummy” hearing aid and ear template at the ment at second-stage Nagata type reconstruction using a
future site of autogenous ear reconstruction. dummy hearing aid.

crucial both for hearing and to avoid scarring the performed implantation on patients both before
site of future autogenous reconstruction (Fig. 7). and after autogenous reconstruction on average
The reconstructive surgeon would like the bone- 5.6 cm from the pseudomeatus, with no difference
anchored hearing aid placed posterior to its nor- in complications; however, both procedures were
mal position. The otologist has concerns that the performed by the same surgeon (G.H.W.). The
bone-anchored hearing aid will pick up sound from pediatric otologist would prefer that the bone-
behind the patient not contributing to binaural anchored hearing aid be sited and implanted as
hearing. A true team approach will alleviate both
surgeons’ concerns. Studies addressing this are
few.76 One promotes an optimal positioning of 6.5
to 7 cm posterior to the auditory meatus. We have

Fig. 8. The patient who had a bone-anchored hearing aid placed at


age 5 years using the technique as illustrated in Figure 7 and later Fig. 10. The same patient following successful autogenous ear
Nagata-type reconstruction of conchal type microtia at age 8 years. reconstruction and fitting with the bone-anchored hearing aid.

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Plastic and Reconstructive Surgery • September 2014

Fig. 11. Ear carving training models that come apart into the various pieces that need to be carved
from costal cartilage. These can be used for training or sterilized and used as a guide intraoperatively.

early as possible (presently, at approximately age 5 LEARNING TO PERFORM EAR


years) to optimize hearing development (Fig. 8). RECONSTRUCTION
The plastic surgeon would prefer that the bone-
Learning to perform ear reconstruction is a
anchored hearing aid implantation be performed
after autogenous reconstruction (age 8 to 10 challenge.77–81 The major emphasis has been on
years) to not compromise the autogenous recon- learning to carve an ear framework from costal
struction (Figs. 9 and 10). The successful use of a cartilage. More recently, the appropriate treat-
bone-anchored hearing aid after a porous polyeth- ment of the soft tissues has been emphasized as
ylene reconstruction has also been reported.50 a major contributing factor to the ultimate aes-
thetic result.

Fig. 12. Model of right sixth, seventh, and eighth rib cartilage
made from a dental impression material used to practice carv- Fig. 13. Carved ear framework using the costal cartilage model
ing an ear framework. shown in Figure 12.

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Volume 134, Number 3 • Microtia Reconstruction

Some type of “observership” and then “giv- hurdles remain.86–92 No material presently avail-
ing it a go” has been a very common approach. able, either autogenous or alloplastic, completely
The “I tried a few but gave up because my results mimics the characteristics of auricular cartilage
weren’t very good” approach is no longer accept- and can produce a normal appearing and func-
able. Historical training methods have been poor tioning reconstructed ear.
or lacked realism. Although carving a vegetable, a The first problem is to create a three-dimen-
piece of soap, or a piece of foam tests the artistic sional scaffold onto which cartilage cells can
ability of the participant, cartilage from the sixth, anchor and subsequently grow. Biological scaf-
seventh, and eighth ribs presents unique chal- folds mimic cartilage extracellular matrix. Natu-
lenges of shape, form, and consistency, requiring ral materials that have been explored include
careful intraoperative decisions that are often not hydrogel, hyaluronic acid, chitosan, and collagen
reversible. Using cadaver cartilage is not practi- derivatives. Their limitations are poor mechani-
cal because it is usually calcified, stiff, and brittle, cal strength, fast degradation, and antigenicity.
not simulating the real surgical circumstance. Synthetic polymers have the advantage of being
Possible disease transmission is also an issue. The custom-made, whereby the biological and mate-
development of more realistic ear carving mod- rial properties can be controlled. However, they
els (Figs. 11 through 13) are allowing surgeons do elicit an immunologic response and lack the
to test their aptitude and gain experience before surface characteristics favoring cellular attach-
performing an actual reconstruction. (See Video, ment and growth. Attempts are being made with
Supplemental Digital Content 4, which displays surface modifications to optimize chondrocyte-
the tutorial and demonstration of a Nagata frame- scaffold interaction. Multiple polymers have been
work. This video is available in the “Related Vid- trialed and produced cartilage formation with
eos” section of the full-text article on PRSJournal. variable loss of shape. Strides have been made in
com or, for Ovid users, at http://links.lww.com/ the precision design and construction of auricular
PRS/B58.) Wilkes and Guilfoyle82 have produced molds based on the normal contralateral ear. The
a training app available at iTunesU, and Chen has use of three-dimensional computer-aided design/
a training model demonstration on YouTube.83 manufacturing has helped make very accurate
Firmin has also developed a “trainer” for learning scaffolds. The designer can control porosity,
ear reconstruction.14 shape, and permeability.
Chondrocytes, both autogenous and xeno-
genic, have been used as the main cell source for
TISSUE ENGINEERING auricular cartilage engineering. One hundred mil-
Excellent reviews of the current state of tissue lion cells are needed to create an adult ear. Auricu-
engineering in auricular cartilage reconstruction lar and nasal chondrocytes yield more cartilage at
were published in 2012.84,85 Tissue engineering a faster rate than articular cartilage. They also have
offers great promise for the future, but major superior histologic and biochemical properties.

Video 4. Supplemental Digital Content 4, which displays the tuto-


rial and demonstration of a Nagata framework, is available in the
“Related Videos” section of the full-text article on PRSJournal.com or,
for Ovid users, at http://links.lww.com/PRS/B58.

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Plastic and Reconstructive Surgery • September 2014

The continued problems remain of dedifferentia-


tion of cells, limited donor supply, and short time
frame for proliferation. The use of chondrogenic
stem cells from bone marrow, periosteum, and adi-
pose is being explored. Managing the cell cultures
through pathways not fully understood has proven
challenging. Inducing factors to stimulate carti-
lage formation include growth factors and bioreac-
tors. Uncontrolled cell proliferation and potential
tumor growth are still concerns.
The use of new “smart” scaffolds of nanocom-
posite polymers with surface modifications to stim-
ulate and control cell attachment, growth, and
differentiation and populated with stem cells may
be the way of the future. The problems of skin cov-
erage of the framework and its effect on the ulti-
mate aesthetics will still prove to be challenging.
Clinical application is in its infancy. Four
patients underwent reconstruction using a two-
Fig. 14. Carved ear framework from tissue-engineered cartilage. stage approach, with cultured chondrocytes
(Used with permission from Yanaga H, Imai K, Fujimoto T, Yanaga injected into the abdominal wall, forming a
K. Generating ears from cultured autologous auricular chondro-
mature block of cartilage.93 In a second stage,
cytes by using two-stage implantation in treatment of microtia.
an ear framework was created. No absorption
Plast Reconstr Surg. 2009;124:817–825.)
of chondrocytes was observed (Figs. 14 and 15).
Composite tissue allotransplantation of an ear has
not been reported, but the anatomical and techni-
cal aspects of harvesting the auricle as a neurovas-
cular facial subunit have been described.94

CONCLUSIONS
The future of ear reconstruction rests with
tissue engineering and possibly composite tissue
allotransplantation. Hearing restoration will be
achieved with completely implantable hearing
devices or new surgical techniques. When these
goals are attained, current approaches described
will be rendered obsolete.
Gordon H. Wilkes, M.D.
University of Alberta
No. 174 Meadowlark Health Center
156 Street and 87 Avenue
Edmonton, Alberta T5R 5W9, Canada
gordon.wilkes@albertahealthservices.ca

PATIENT CONSENT
Fig. 15. Result of microtia surgery using tissue-engineered Patients provided written consent for the use of their
cartilage 4 years 6 months after implantation into the images.
temporal area. (Used with permission from Yanaga H, Imai
K, Fujimoto T, Yanaga K. Generating ears from cultured
autologous auricular chondrocytes by using two-stage ACKNOWLEDGMENTS
implantation in treatment of microtia. Plast Reconstr Surg. The authors thank Kathy Bush for administrative
2009;124:817–825.) assistance and Farzine MacRae for film editing.

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Volume 134, Number 3 • Microtia Reconstruction

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