Sensorineural Hearing Loss

감각신경성 난청

분당 차병원 이비인후과 부교수 이창호

 SNHL = Cochlear HL
• Sensorineural Hearing Loss
– Cochlear(Sensory) HL

• Neural HL 빈도상 적음
– Auditory Neuropathy
– Central HL
 Cochlear HL = Hair Cell damage
• Hair bundles demonstrate slight irregularities in stereocilia lengths within the characteristically packed
rows of the hair bundle. Arrows pointing down show slightly shorter stereocilia, whereas arrows
pointing up show longer stereocilia in the same row.
• Figure 2. The mammalian organ of Corti before and after hair-cell loss and a
• speculative visualization of potential regeneration of hair cells. (a) A cross-section
• of the normal organ of Corti usually bears a single inner hair cell and three outer
• hair cells. All hair cells are depicted in green. (b) After hair-cell loss, the organ of
• Corti undergoes morphological rearrangement. In some cases, the remaining cells
• accrue to reseal the epithelium; often the cells lose their morphological features
• and adopt a basic epithelial organization, as depicted here. (c) Hypothetical view of
• a regenerated sensory epithelium in place of the degenerated organ of Corti. New
• hair cells (green) can either be reseeded by the integration of progenitor cells or
• they can differentiate, following suitable stimulation, by direct regeneration from
• the cells that remain after hair-cell loss.
 SNHL = HC damage
• Hair cell damage 가 있다 . 없다 .
 학습목표 SNHL
• Congenital SNHL 선천성난청
• Presbycusis 노인성난청
• Noise induced HL 소음성난청
• Ototoxicity 이독성난청

• Sudden SNHL 돌발성난청

• Acoustic Neuroma 청신경종양
– Retrocochlear lesion
 Diagnosis of SNHL
• Purely by Audiometry
– See Audiometry section

• Normal TM
– Absent P/E finding
NIHL - Noise Induced HL
 Cochlea – ScanningEM sutrcture
• P.155 그림 73
 Noise induced HL (NIHL) - Overview

• Very common , = Occupational HL

• Acoustic trauma
• TTS Temporary threshold shift
• PTS Permanent threshold shift

• Affects high frequencies first (4 kHz); often progressive

• Frequently associated with tinnitus
• OHC injury  IHC loss  Cochlear nuclues degeneration

• No known treatment. Counsel patient to avoid noise in future

• PREVENTION is key to reducing incidence
4000 kHz C5 Dip in NIHL
 Protection from Noise
• Tensor Tympani

• Stapedius m

• Stapedial Reflex
– Input : CN8
– Output : CN7 = Facial nerve branch to stapedius
 Sudden SNHL
• Definition
– >30(20)dB of hearing loss
– over at least 3 contiguous audiometric
frequencies
– occurring within 3 days or less
cf. “rapidly progressive” for HL over more than 3 days.

• Sudden sensorineural hearing loss (SSNHL) is a

medical urgency.
• Admission for Medical treatment (steroid etc) and
BR
 SSNHL - Frightening experience for patients

• Organ of Corti
• Frightening experience of the patient
– The patients might assume that this is a life threatening disorder or will
lead to profound, bilateral deafness
 Vestibular schwannoma
= Acoustic neuroma
• 1YA Dizziness with illusinon of motion
– Nonwhiling type
– Headache
– Severe complaint
• 1YA AMC : L SNHL

• 2004-6-30 CHAMC ENT OPD

• 2004-7-7
– L 50/50
– Head shake nystagmus to R
• 2004-7-10 TB MR
– L Acoustic neuroma
 Temporal Bone MR
• T2
 Pediatirc SNHL
• Congenital
– Genetic SNHL
– Nongenetic SNHL

• Acquired
– Meningitis : H.influenza
.EHDI
 EHDI
• EHDI
– Early Hearing Detection and Intervention Program
• UNHS
– Universal Newborn Hearing Screening
– 전신생아 청력 선별
• HRR
– High risk register
 HRR High Risk Register ( 주관식 )
• Family history of hereditary childhood sensorineural hearing loss
• In utero infection TORCH(eg, rubella, cytomegalovirus, syphilis, toxoplasmosis,
herpes)
• Craniofacial anomalies
• Low birth weight (<1500 g or 3.3 lb)
• Hyperbilirubinemia at levels requiring exchange transfusion
• Bacterial meningitis
• Exposure to ototoxic medications
• Mechanical ventilation lasting 5 days or longer
• Stigmata or other findings associated with a syndrome known to include a
sensorineural and/or conductive hearing loss
• Apgar scores of 0-4 at 1 minute or 0-6 at 5 minutes
 EHDI Basics
• Each year in the United States (U.S.), more than 12,000 babies are born with a hearing loss. The cause
of hearing loss for many babies is not known, and hearing loss can go unnoticed for years.

• Studies have shown that children who have a hearing loss can have delays in speech, language, social
skills, and academic achievement. This is why all babies need a hearing screening, which helps find
children who may have a hearing loss.

• Most babies have a hearing screening soon after birth, usually before they leave the hospital. This is
often called Universal Newborn Hearing Screening (UNHS). That means everybody is screened.

• When a child’s hearing loss is identified soon after birth, the child’s family and doctors can make sure
the child gets services (e.g., intervention) he or she needs at an early age. This will help the child
develop communication and language skills that will last a lifetime.
 EHDI Basics
• Early Hearing Detection and Intervention (EHDI) programs are set up by U.S. states
and territories to help make sure that infants and children with hearing loss are found
and receive help (e.g., intervention) as soon as possible.

• UNHS is the first step in the EHDI process; other important steps are audiological
evaluation to confirm a hearing loss, and early intervention services.
• The EHDI program at CDC supports states and territories in developing and
implementing EHDI tracking and surveillance systems. These systems help state EHDI
programs make sure that babies get the hearing screening, follow-up, and early
intervention services they
•
 Neonatal screening in UNHSP
• UNHSP = Universal newborn hearing screening project
• Detect deaf or SNHL on birth ~ before 3 months
• Objective test + Fast and reliable test

• 1. aABR ; ALGO
• 2. OAE
•
• --> Confirm with ABR threshold testing with sedation
•
 유소아 청력검사
– BOA: Behavioral observation audiometry
– Play audiometry

• Objective audiometry
• ABR (Auditory Brainstem Response) with threshold
– Sedation in Audio-Booth
– Confirmative tool
• OAE Otoacoustic emission
– Fast test time
– Screening
• aABR
– Fast ABR ALGO for Screening
 BERA

• BERA Brainstem auditory response audiometry

• = BAEP, Brainstem auditory evoked potential

1. Threshold testing
– Confirmative objective test (children, Malingering)
– aABR (automated) = Fast ABR
2. Latency testing
– Retrocochlear lesion dx (VS)
BERA Fig
 Otoacoustic emission
• Cochlear origin, especially OHC origin
• Objective test
• Fast but not reliable
– (+) if HL < 30dB, (+) means Good hearing
– (-) if HL > 40dB
• False (-) if conductive HL, noisy environment = crying baby
• So (-) means nothing

Useful for screening

– Neonatal screening - very fast, but false negative
– NIHL screening
– Ototoxicity screening (Chemotherapy)
 Hair Cell damage
• Hair bundles demonstrate slight irregularities in stereocilia lengths within the characteristically packed
rows of the hair bundle. Arrows pointing down show slightly shorter stereocilia, whereas arrows
pointing up show longer stereocilia in the same row.
• Figure 2. The mammalian organ of Corti before and after hair-cell loss and a
• speculative visualization of potential regeneration of hair cells. (a) A cross-section
• of the normal organ of Corti usually bears a single inner hair cell and three outer
• hair cells. All hair cells are depicted in green. (b) After hair-cell loss, the organ of
• Corti undergoes morphological rearrangement. In some cases, the remaining cells
• accrue to reseal the epithelium; often the cells lose their morphological features
• and adopt a basic epithelial organization, as depicted here. (c) Hypothetical view of
• a regenerated sensory epithelium in place of the degenerated organ of Corti. New
• hair cells (green) can either be reseeded by the integration of progenitor cells or
• they can differentiate, following suitable stimulation, by direct regeneration from
• the cells that remain after hair-cell loss.
Otoacoustic Emissions
Otoacoustic Emission

SOAE

TEOAE
DPAOE
 Distortion Product OAE - WNL

•Auditory
thresholds 0-5dB HL,
each ear; patient
is 41 year old
female
 DPOAE with Hearing Loss

52 yr male; Audio results

Left Right
2K: 15 2K: 30
3K: 35 3K: 80
4K: 35 4K: 85
6K: 40 6K: 60
8K: 15 8K: 60
 Objective audiometry
• Pediatric evaluation or DDx malingering

• Impedance audiometry (Stapedial reflex)

– Suprathreshold reflex only
• Electrocochleography 전기와우청력검사
– invasive
• Evoked OAE (Otoacoustic emission) 유발이음향방사검사
• BERA (Brainstem evoked response audiometry) 뇌간유발반응검사
 Tests for Malingering
• 반복검사
– PTA, SRT, SDS, Bekesy audiometry
• Objective test
• Others
– Strenger, Doerfler Stewart, Lombard, Delayed feedback, Electrodermal
 Hearing aid vs Cochlear
Implantation
불필요
학습목표

부분적으로 필
요

빈번한 필요

항상 필요

항상 필요 , 미온적인 도움

특수보청기 , 인공와우 대상
 학습목표 - 음의 전달 경로중 옳은 것은 ?

• ① 외이도 - 고막 -malleus-incus-stapes-
oval window-organ of corti 
• ② round window-scala tympani-scala
media-scala vestibuli
• ③ 외이도 - 고막 -incus-malleus-round
window-organ of corti
• ④ oval window-scala media-scala tympani-
scala vestbuli
 How we hear
H
OT
RL S
S N
S N
N
IP OP
I
B IT D
IPh

Cochlea : analyzer Hydraulic energy

Organ of Corti : OHCs & IHCs
 교과서의 조악한 그림
• 학생 교과서 53
Osseous Labyrinth FIg
 2 window in Cochlea

• Cochlea = Labyrinth

• Bony labyrinth
– Perilymph outside cochlear duct (scala media)
• Membraneous labyrinth
– Endolymph in cochlear duct

• Ovall window
– Stapes in OW
• Round window
 How We Hear - Sequence
• Middle ear
– Malleus, Incus, Stapes
• Stapes in Oval window
• Perilymph
– Scala vestibuli to Scala tympani
• Basilar membrane
• Reissner’s membrane
• Endolymph (in Cochlear duct)
• Organ of Corti (OHC  IHC)
 학습목표

• 고음역 청력장애를 일으키는 것은 주로 어느 부

분의 장애 때문인가 ?
How to discriminate frequency
 Organ of Corti in Cochlea
• Fig. 2. Anatomy of the adult mammalian cochlea. (A) Cross-section through the human head,
illustrating the position of the inner ear. The ear has three parts: the external ear, which consists of
the pinna and external auditory meatus (EAM) and ends at the tympanic membrane (TM, green); the
middle ear (ME, orange), which contains the three middle ear bones; and the inner ear, which includes
the bony labyrinth (BL, light blue) and the statoacoustic ganglion (SAG, purple). (B) Lateral view of the
mouse inner ear (boxed region in A) with sensory epithelia in green. LC, lateral semicircular canal; SC,
superior semicircular canal; PC, posterior semicircular canal; Sac, saccule; Utr, utricle; Coch, cochlea; ES,
endolymphatic sac. The endolymphatic duct is hidden from view but connects the ES to the
semicircular canals. (C) Cross-section through the boxed region in B, illustrating the anatomy of the
cochlear duct. The duct is separated into scala vestibule (SV), scala media (SM) and scala tympani (ST).
The sensory epithelium of the cochlea, the organ of Corti (boxed), is located on the floor of the scala
media. (D) Cross-section of the organ of Corti, containing a single inner hair cell (I, green), three outer
hair cells (1-3, green) and non-sensory supporting cells (blue), which include the pillar cells (purple).
Inner and outer sensory hair cells are separated by the tunnel of Corti (TC), a fluid filled structure that
is bounded by inner and outer pillar cells (IP and OP, purple).
Cochlea – Organ of Corti
Summary of Passive Cochlear Mechanics
• Basilar membrane
: space of Nuel 과 basilar memb. 을 통한 perilymph
의 diffusion 에 의해 Corti organ 의 metabolite 를
교환함
• Organ of Corti
: basilar memb 의 mechanical vibration 을 neural
impulse 로 전환
reticular lamina 를 형성
 Corti’ s organ
• Lateral wall
2. Spiral ligament
3. Stria vascularis : marginal cell 에서 endolymph 생성
4. Spiral prominence : ion transport
5. External sulcus
• Sensory cell
1. Stereocilia
: apex 로 갈수록 길어진다
outer hair cell 의 stereocilia 만 tectorial memb. 에 부
착된다
2. Outer haircell
3. Inner haircell
Cochlear potential
K+ Flow
Potentials in the Cochlea

Perilymph Endolymph
154Na+ 1
3 K+ 161
128 Cl- 131

S. Media : + 80 mV
OHC : - 70 mV
IHC : - 40 mV
 Hair Cell damage
• Hair bundles demonstrate slight irregularities in stereocilia lengths within the characteristically packed
rows of the hair bundle. Arrows pointing down show slightly shorter stereocilia, whereas arrows
pointing up show longer stereocilia in the same row.
• Figure 2. The mammalian organ of Corti before and after hair-cell loss and a
• speculative visualization of potential regeneration of hair cells. (a) A cross-section
• of the normal organ of Corti usually bears a single inner hair cell and three outer
• hair cells. All hair cells are depicted in green. (b) After hair-cell loss, the organ of
• Corti undergoes morphological rearrangement. In some cases, the remaining cells
• accrue to reseal the epithelium; often the cells lose their morphological features
• and adopt a basic epithelial organization, as depicted here. (c) Hypothetical view of
• a regenerated sensory epithelium in place of the degenerated organ of Corti. New
• hair cells (green) can either be reseeded by the integration of progenitor cells or
• they can differentiate, following suitable stimulation, by direct regeneration from
• the cells that remain after hair-cell loss.
 Tuning curve
• Inner hair cell : frequency
• Outer hair cell : threshold
 Tuning curve
• Tone burst 시에 그에 해당하는 부위 의 auditory
nerve 가 V -shape 을 그리게 되는 데 이 tip 은 vest
identified frequency 나 characteristic frequency 를 나타내
게 된다 .
IHC 손상 , OHC 손상
 IHC 손상 , OHC 정상
: tip 유지되고 threshold 만 30dB 상승
 IHC 정상 ,OHC 부분손상
: monoaural diplacusis 발생
IHC 정상 , OHC 손상
: threshold shift 40 dB
END