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Evaluation of a Deaf Child — ENT Perspective

KUHS Final MBBS | 10-Mark Format

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🔑 Opening Hook (What Examiners Love)

"Deafness in a child is a communication emergency — every month of delay costs language." The goal is to detect early, diagnose accurately, and rehabilitate promptly. The ideal benchmark: 1-3-6 rule — Screen by 1 month, Diagnose by 3 months, Intervene by 6 months.

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I. DEFINITION & PREVALENCE

• Congenital hearing loss affects 1–3 per 1000 newborns (most common sensory birth defect)
• Without universal newborn hearing screening (UNHS), children are identified only when speech delay becomes obvious — often as late as 2–3 years

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II. HISTORY (4 marks potential here)

A. Presenting Complaint

• Age at which parents first noticed lack of response to sound
• Lack of speech development, not startling to loud noises, not turning to mother's voice

B. Antenatal History (TORCH + Risk factors)

C. Perinatal History

• Prematurity, birth asphyxia, neonatal jaundice (hyperbilirubinemia → kernicterus)
• NICU stay > 5 days, exchange transfusion, mechanical ventilation

D. Postnatal History

• Meningitis ⭐ (most common cause of acquired SNHL in children)
• Mumps, measles
• Head trauma
• Ototoxic drug use (gentamicin, furosemide)

E. Developmental History

• Speech milestones — cooing (2 months), babbling (6 months), single words (1 year), 2-word phrases (2 years)
• School performance, reading difficulties

F. Family History

• Autosomal recessive deafness is most common (GJB2 gene, Connexin 26)
• Autosomal dominant (Waardenburg syndrome, branchio-oto-renal syndrome)
• X-linked (Norrie's disease, Alport syndrome)

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III. PHYSICAL EXAMINATION

A. General Examination — "Look for Syndromes"

B. ENT Examination

1. Pinna — microtia, bat ear, preauricular sinus/tag, skin tags
2. External auditory canal — atresia, stenosis, wax impaction
3. Tympanic membrane — perforation, retraction, glue ear (otitis media with effusion — most common cause of CHL in children)
4. Nose and throat — adenoid hypertrophy (causes OME)
5. Palate — cleft palate (associated with OME)

C. Neurological Exam

• Eye movements, balance (rule out Usher syndrome, labyrinthine dysfunction)

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IV. AUDIOLOGICAL INVESTIGATIONS ⭐ (The Crux)

0–6 Months: Objective Tests

⭐ ABR is the gold standard for confirming hearing loss in infants under 6 months — performed during natural sleep

6 Months – 2.5 Years: Behavioral Tests

≥ 5 Years: Pure Tone Audiometry (PTA)

• Full conventional audiogram: air conduction (250–8000 Hz) + bone conduction
• Determines: Type (conductive/SNHL/mixed), degree, configuration of loss
• Speech Audiometry: SRT (Speech Reception Threshold), SDS (Speech Discrimination Score)

Additional Tests

• ASSR (Auditory Steady State Response) — frequency-specific thresholds in sleeping infants
• Acoustic Reflexes (stapedial reflex) — absent in severe SNHL, elevated in CHL

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V. RADIOLOGICAL INVESTIGATIONS

⭐ MRI is mandatory before cochlear implantation to confirm cochlear nerve presence.

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VI. LABORATORY / SPECIAL INVESTIGATIONS

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VII. CLASSIFICATION OF HEARING LOSS (Quick Reference)

• Conductive — Outer/Middle ear (OME, wax, atresia) — Air-bone gap present
• Sensorineural — Cochlea/8th nerve (genetic, CMV, meningitis)
• Mixed — Both

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VIII. MANAGEMENT OUTLINE (for completeness)

• CHL: Treat cause (grommets for OME, surgery for atresia)
• Mild–moderate SNHL: Hearing aids (BTE — behind-the-ear preferred in children)
• Severe–profound SNHL: Cochlear implant — best outcomes if done before 2 years of age
• Auditory verbal therapy (AVT) and special education regardless of type
• Genetic counselling for families

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✍️ Examiner-Friendly Summary Table

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🎯 Golden Points to Score Full Marks

1. 1-3-6 rule — examiners love this
2. OAE screens, ABR confirms — the standard sentence
3. Age-appropriate audiological tests — BOA → VRA → CPA → PTA
4. Jervell-Lange-Nielsen = SNHL + Long QT (ECG mandatory — life-threatening!)
5. Connexin 26 (GJB2) = most common genetic cause
6. CMV = most common congenital viral cause
7. Meningitis = most common cause of acquired SNHL
8. Glue ear/OME = most common cause of conductive hearing loss in children
9. MRI mandatory before cochlear implant (to confirm cochlear nerve)
10. Cochlear implant before age 2 → best language outcomes

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TINNITUS — ENT

Perfect 10-Mark Answer | KUHS Final MBBS Format

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🔑 Opening Line (Hook the Examiner)

"Tinnitus is the perception of sound in the absence of an external acoustic stimulus." It is a symptom, not a disease — and the examiner's job (and yours) is to find the underlying cause.

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I. DEFINITION

• Affects approximately 10–15% of the general population
• Approximately 20% find it bothersome enough to seek treatment
• Can cause: insomnia, impaired concentration, depression, anxiety, impaired work performance

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II. CLASSIFICATION ⭐ (Examiners always test this)

A. Based on Audibility

B. Based on Rhythm

C. Based on Etiology

⭐ Subjective nonpulsatile = 90% of all ENT tinnitus referrals — the most common type

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III. ETIOLOGY / CAUSES ⭐

A. Subjective Tinnitus

• Cerumen impaction / Foreign body — external ear
• Otitis media with effusion, otosclerosis — middle ear
• SNHL — presbycusis ⭐ (most common overall), noise-induced hearing loss
• Ménière's disease — low-pitched roaring tinnitus + fluctuating SNHL + vertigo
• Vestibular schwannoma (Acoustic neuroma) — unilateral tinnitus ⭐ red flag
• Ototoxic drugs — aspirin, NSAIDs, aminoglycosides, loop diuretics (furosemide), quinine, cisplatin

• Hyperthyroidism, hypothyroidism
• Hyperlipidaemia
• Anaemia
• Vitamin B12 / zinc deficiency

• Multiple sclerosis
• Basilar skull fracture, whiplash injury

• Aspirin / NSAIDs — reversible, dose-dependent
• Aminoglycosides — gentamicin (vestibulotoxic), tobramycin, streptomycin (cochleotoxic)
• Loop diuretics — furosemide (especially combined with aminoglycosides)
• Quinine / chloroquine
• Cisplatin — high-frequency SNHL
• Tricyclic antidepressants, oral contraceptives, caffeine

• Temporomandibular joint (TMJ) dysfunction
• Psychological — depression, anxiety (somatosensory tinnitus)

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B. Objective Tinnitus (Causes — all vascular or mechanical)

⭐ Palatomyoclonus = clicking tinnitus — rhythmic, heard by examiner, caused by clonic contractions of palatal muscles (tensor/levator veli palatini)

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IV. CLINICAL FEATURES / HISTORY TAKING

Key Questions to Ask:

1. Laterality — unilateral or bilateral?
   • Unilateral → Red flag (vestibular schwannoma, vascular cause)
   • Bilateral → Usually benign SNHL
2. Quality — ringing? roaring? clicking? pulsatile?
3. Duration — acute (<3 months) vs chronic (>6 months)
4. Aggravating/relieving factors — position change (vascular), jaw movement (TMJ)
5. Associated symptoms — hearing loss, vertigo, headache, facial numbness
6. Drug history — aspirin, aminoglycosides, furosemide
7. Noise exposure — occupational or recreational
8. Systemic symptoms — palpitations (anaemia, thyrotoxicosis)

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V. EXAMINATION

General

• Anaemia (pallor), goitre (thyrotoxicosis)
• BP measurement (hypertension)

ENT Examination

1. Auroscopy — wax, OME, retrotympanic pulsatile mass (glomus tumour — reddish-blue pulsating mass)
2. Auscultation — periauricular region, neck (for bruit), chest (murmur)
3. Palate movement — clicking palate = palatomyoclonus
4. TMJ — clicking jaw = TMJ dysfunction

Neurological

• Cranial nerve exam (CN V, VII, VIII) — for cerebellopontine angle lesions

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VI. INVESTIGATIONS

Audiological (Mandatory in all cases)

Imaging ⭐ (Know exactly when to order what)

Laboratory Tests

• CBC — anaemia
• Thyroid function tests — hypo/hyperthyroidism
• Fasting lipid profile — hyperlipidaemia
• Blood glucose — diabetes
• VDRL/FTA-ABS — syphilis (luetic labyrinthitis)

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VII. MANAGEMENT ⭐ (Best marks come here)

Step 1: Treat the Underlying Cause

• Remove impacted wax
• Treat OME (grommets)
• Stop ototoxic drugs
• Correct anaemia, thyroid disorders

Step 2: Hearing Rehabilitation

• Hearing aids — amplification reduces tinnitus perception in patients with SNHL; most effective treatment for tinnitus with hearing loss
• Cochlear implants — severe-profound SNHL with tinnitus

Step 3: Tinnitus-Specific Therapies

Step 4: Pharmacological

⭐ No drug is FDA-approved specifically for tinnitus.

⭐ Avoid rTMS (repetitive transcranial magnetic stimulation) and TENS — no proven benefit

Step 5: Surgical (for specific causes)

• Glomus tumour — surgical excision / embolization / radiotherapy
• Vestibular schwannoma — surgery / stereotactic radiosurgery (Gamma Knife)
• Carotid artery stenosis — carotid endarterectomy
• Superior semicircular canal dehiscence — surgical plugging
• Palatomyoclonus — botulinum toxin injection into palatal muscles

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VIII. RED FLAGS — Refer / Investigate Urgently ⭐

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IX. IMPORTANT POINTS FOR DISTINCTION (Examiner's Traps) ⭐

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✍️ Examiner-Favourite Summary

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🎯 Golden Lines to Write in Exam

1. "Tinnitus is a symptom, not a disease — the aim is to identify the cause."
2. "Unilateral tinnitus with asymmetric SNHL mandates MRI to exclude vestibular schwannoma."
3. "Pulsatile tinnitus with a normal tympanic membrane requires MRA to rule out vascular pathology."
4. "A reddish-blue pulsatile mass behind the tympanic membrane = glomus tumour — do NOT biopsy in the clinic."
5. "TRT (Tinnitus Retraining Therapy) combining directive counselling with sound therapy offers the best long-term habituation."
6. "No medication has been FDA-approved specifically for tinnitus; management is primarily rehabilitative."

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