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Hearing Loss - Complete Overview
1. History of Hearing Loss (H/O)
A thorough history is the cornerstone of evaluating any patient with hearing loss. The following key points must be elicited:
Key History Points
Characteristics of the hearing loss:
- Duration and age of onset - congenital vs. acquired
- Rate of progression - sudden onset vs. gradual/insidious
- Pattern - stable, slowly progressive, or fluctuating
- Laterality - unilateral vs. bilateral
- Better-hearing ear - always ask which ear hears better
Associated symptoms:
- Tinnitus (ringing in the ears)
- Vertigo or imbalance
- Aural fullness
- Otalgia (ear pain)
- Otorrhea (ear discharge)
- Headache
- Facial nerve dysfunction
- Head and neck paresthesias
Relevant background history:
- History of head trauma
- Exposure to ototoxic drugs (aminoglycosides, loop diuretics, cisplatin, quinine)
- Occupational or recreational noise exposure
- Previous ear surgery
- Prior use of hearing aids
Family history:
- Family history of hearing loss or ear surgery
- In children, additionally: gestational, perinatal, and postnatal history; syndromic associations (renal, cardiac, craniofacial, neurological, metabolic disorders)
Signs and symptoms suggestive of hearing loss (Table 36-2, Harrison's):
- Frequently saying "huh?"
- Reduced clarity of hearing
- Difficulty understanding in background noise
- Family members complaining about the patient's hearing
- Turning up volume on TV/radio
- Avoiding social settings
- Sensitivity to loud noises
2. Clinical Features of Hearing Loss
Types of Hearing Loss
| Type | Key Feature |
|---|
| Conductive | Problem in outer or middle ear; bone conduction > air conduction (Rinne negative) |
| Sensorineural (SNHL) | Problem in cochlea or auditory nerve; air conduction > bone conduction in both ears |
| Mixed | Both components present |
Clinical Pointers by Cause
- Sudden unilateral loss + tinnitus → viral labyrinthitis, vestibular schwannoma, stroke
- Gradual progressive loss → otosclerosis, noise-induced hearing loss, vestibular schwannoma, Meniere's disease
- Fluctuating loss + episodic vertigo + tinnitus + aural fullness → Meniere's disease
- Sound-induced vertigo + autophony + hearing own eye movements → superior semicircular canal dehiscence
- Hearing loss + otorrhea → chronic otitis media or cholesteatoma
- Unilateral serous effusion → must exclude nasopharyngeal carcinoma
- Bilateral loss + renal disease → Alport's syndrome
- Loss + retinitis pigmentosa → Usher's syndrome
- Loss + pigmentary changes → Waardenburg's syndrome
- Loss + goitre → Pendred's syndrome
- Loss + prolonged QT → Jervell and Lange-Nielsen syndrome
Tuning Fork Tests
Rinne Test (512 Hz):
- Normal / SNHL: Air conduction (AC) > Bone conduction (BC) - Rinne positive
- Conductive HL ≥ 30 dB: BC > AC - Rinne negative
Weber Test:
- Unilateral conductive loss: sound lateralizes to the affected ear
- Unilateral SNHL: sound lateralizes to the unaffected (better) ear
- A 5 dB difference between ears is needed for lateralization
3. Hearing Assessment
Minimum Audiologic Battery (Harrison's, p. 1307)
A complete hearing assessment should include:
| Test | Purpose |
|---|
| Pure tone audiometry (PTA) | Establishes air- and bone-conduction thresholds (250-8000 Hz); determines type and degree of loss |
| Speech reception threshold (SRT) | Lowest intensity at which patient correctly repeats 50% of spondee words |
| Word recognition score (WRS) | % of words correctly identified at suprathreshold levels |
| Tympanometry | Middle ear pressure, compliance, Eustachian tube function |
| Acoustic reflexes | Stapedial reflex; differentiates cochlear from retrocochlear loss |
| Acoustic reflex decay | Suggests retrocochlear (VIIIth nerve) pathology if present |
Degrees of Hearing Impairment
| PTA Threshold | Degree |
|---|
| 0-25 dB | Normal |
| 26-40 dB | Mild |
| 41-55 dB | Moderate |
| 56-70 dB | Moderately severe |
| 71-90 dB | Severe |
| >90 dB | Profound |
Additional Tests
- Otoacoustic Emissions (OAEs) - assess outer hair cell (OHC) function; present in conductive loss, absent/reduced in cochlear SNHL; used in newborn screening
- TEOAEs (transient evoked) - click-stimulated; present if response ≥ 4 dB above noise floor in at least 3 octave bands
- DPOAEs (distortion product) - frequency-specific OHC testing using two continuous tones (f1, f2); largest DPOAE at 2f1-f2
- Auditory Brainstem Response (ABR) - electrophysiological; used in neonates, non-cooperative patients, retrocochlear pathology; prolonged I-V latency suggests retrocochlear disease
- Electronystagmography / VEMP - for vestibular component
- CT/MRI temporal bone - structural pathology, cholesteatoma, vestibular schwannoma
- Genetic testing - connexin 26 (GJB2), GJB6 mutations in congenital/hereditary SNHL
4. Management of Hearing Loss
Management depends on the type, severity, and etiology of hearing loss.
A. Conductive Hearing Loss
| Cause | Management |
|---|
| Cerumen impaction | Removal (suction, cerumenolytics, irrigation - avoid irrigation if TM perforation) |
| Otitis media with effusion | Conservative (3 months); then ventilation tubes (grommets) |
| Otitis externa | Topical antibiotics, aural toilet |
| Tympanic membrane perforation | Tympanoplasty |
| Ossicular discontinuity | Ossiculoplasty |
| Otosclerosis | Stapedectomy / stapedotomy; fluoride therapy; hearing aid |
| Cholesteatoma | Mastoidectomy + ossicular reconstruction |
B. Sensorineural Hearing Loss
| Type | Management |
|---|
| Sudden SNHL | Systemic/intratympanic corticosteroids (within 4-6 weeks of onset) |
| Noise-induced | Prevention (ear protection); no curative treatment; hearing aids |
| Presbycusis | Hearing aids; auditory rehabilitation |
| Meniere's disease | Low-salt diet, diuretics, betahistine; intratympanic gentamicin/steroids; endolymphatic sac surgery |
| Ototoxicity | Stop offending drug if possible; monitor; hearing aids |
| Autoimmune inner ear disease | Systemic steroids, immunosuppressants |
C. Hearing Rehabilitation Devices
- Conventional hearing aids (BTE, ITE, ITC, CIC types) - for mild to severe loss; recommended from 6 months of age in congenital loss
- Bone-anchored hearing aids (BAHA) - for single-sided deafness or conductive loss in patients unable to use conventional aids
- Cochlear implants (CI) - for profound bilateral SNHL; indicated from age 1-2 years in children with profound SNHL; pre-lingual and post-lingual candidates
- Auditory brainstem implants (ABI) - for bilateral vestibular schwannomas (NF2) where cochlear implant is not possible
D. Communication and Auditory Rehabilitation
- Lip reading / speechreading training
- Sign language (especially for pre-lingual deafness)
- Assistive listening devices (ALDs), FM systems
- Auditory-verbal therapy in children
- Counselling for patients and families
Sources: Harrison's Principles of Internal Medicine 22e, p. 1283-1307; Shambaugh Surgery of the Ear, pp. 3411-3441; Scott-Brown's Otorhinolaryngology Head & Neck Surgery Vol 2; Cummings Otolaryngology Head and Neck Surgery; K.J. Lee's Essential Otolaryngology.