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EN4.{12-14,16-20} | Hearing Vestibular and Neuro Otology — PBL Case

CLINICAL SETTING

Professor Rajesh Nair, a 52-year-old professor of biochemistry, arrives at the ENT outpatient department accompanied by his wife. He has been referred urgently by his GP after two frightening episodes over the past 6 months. The first episode, 6 months ago: he woke at 3 AM with the room violently spinning. He could not walk to the bathroom without holding the wall. He vomited twice. The spinning lasted about 2 hours, then resolved. His wife noticed he seemed to be 'not hearing properly' in his left ear for a day or two after the attack. He had a low-pitched ringing in his left ear throughout. The second episode, 2 weeks ago: identical. This time he noticed his left ear felt 'blocked and full' before the vertigo started — almost like a warning sign. Between episodes he feels well, though his left ear tinnitus persists. Today, you examine him. His general examination is normal. Ear examination: both tympanic membranes appear intact and normal. When you perform the tuning-fork tests, you notice his Rinne test is positive bilaterally, but his Weber lateralises to the RIGHT ear. There is no nystagmus at rest. His wife adds: 'Last night, when I mentioned the appointment, he suddenly looked pale, grabbed the wall, and said the room was starting to spin again. It settled in about 2 minutes when he lay still.'

Trigger 1: Interpreting the Presentation

You complete your initial assessment of Professor Nair. Tuning-fork results: Rinne positive bilaterally (AC > BC on both sides); Weber lateralises to the right. Absolute bone conduction: normal on the right, reduced on the left. There is no nystagmus at rest. Romberg's test: slightly positive (falls to the left with eyes closed). Dix-Hallpike test: negative bilaterally. Fistula test: negative. He has no history of ear discharge, no previous ear surgery, no head injury, no family history of hearing problems, and takes no ototoxic medications.

DISCUSSION POINTS

  • Interpret the tuning-fork findings systematically: What does Rinne positive bilaterally tell you? In which ear does the Weber lateralise, and what does lateralisation to the RIGHT indicate about which ear has pathology — and what type of hearing loss?
  • The Dix-Hallpike test is negative bilaterally. Based on this and the clinical history, which two common diagnoses can now be effectively excluded, and why?
  • Before ordering the audiogram, construct your differential diagnosis in order of probability. What is the single feature in the history that most strongly points to the top diagnosis?
  • Professor Nair asks: 'Is this serious? Will I go deaf?' How do you respond using the principle of calibrated uncertainty — being honest about what you know, what you don't yet know, and the next investigative steps?
Click to reveal Trigger 2: Audiogram and the Face That Wouldn't Move (discuss previous trigger first!)

Trigger 2: Audiogram and the Face That Wouldn't Move

The audiogram confirms: left ear — bilateral symmetrical low-frequency sensorineural hearing loss with thresholds of 40 dB at 250 Hz and 500 Hz, recovering to near-normal at 2000 Hz and above. Right ear — normal. Tympanometry: type A bilaterally (normal compliance, normal peak pressure). Stapedial reflexes: present on the right, absent on the left. Three weeks later, Professor Nair returns urgently. His wife is visibly distressed. This morning he noticed the left side of his face was 'drooping.' On examination: complete left-sided lower motor neurone facial palsy — forehead involvement, inability to close the left eye, absence of the nasolabial fold on the left, and deviation of the mouth to the right when he tries to smile. He also reports loss of taste over the front of his tongue. There is no parotid swelling. He denies any ear pain, but you notice small vesicles around the left pinna and in the external auditory canal.

DISCUSSION POINTS

  • Analyse the audiogram: Is this pattern consistent with the working diagnosis from Trigger 1? What does low-frequency SNHL recovering at higher frequencies indicate about the pathophysiology? How does the absent left stapedial reflex support this?
  • Now focus on the new problem. The facial palsy involves the forehead and there is loss of taste on the anterior tongue. Localise the facial nerve lesion anatomically: Is this upper motor neurone or lower motor neurone? And at what level within the temporal bone, based on the taste loss?
  • Looking at the full clinical picture — vesicles around the left pinna and EAC, LMN facial palsy, altered taste — what is the unifying diagnosis for the NEW problem? Name the syndrome, state its cause, and explain why the ear vesicles are present.
  • What are the immediate management steps for this new facial nerve condition? Which medications are used, what is the time window for maximum effectiveness, and what is the key ophthalmological priority?
Click to reveal Trigger 3: Long-Term Management and Patient Communication (discuss previous trigger first!)

Trigger 3: Long-Term Management and Patient Communication

Six weeks later: Professor Nair's facial palsy has partially recovered (House-Brackmann grade III). He continues to have episodic vertigo attacks from his Meniere's disease — now totalling four attacks in 8 months, each causing him to miss a day of teaching. He has been on a low-salt diet and betahistine 16 mg three times daily for the past 6 weeks. He asks you three questions directly: 1. 'Will my face recover fully?' 2. 'My vertigo is still happening. Is there anything stronger you can do, short of removing my ear?' 3. 'My 26-year-old son was recently found to have some hearing loss. Could he have inherited this condition from me?' The department head has also asked whether Professor Nair's case should be discussed anonymously at the medical ethics review, because he drives to work and his episodic vertigo creates a road safety concern.

DISCUSSION POINTS

  • Professor Nair's facial recovery has reached House-Brackmann grade III at 6 weeks. What does this grade mean clinically? Based on the natural history of Ramsay Hunt syndrome compared to Bell's palsy, how should you counsel him about his long-term prognosis?
  • Medical management of his Meniere's disease has failed to control his attacks adequately. Outline the escalating treatment options available beyond medical therapy. What is the mechanism of intratympanic gentamicin, and what is the main risk the patient must understand before consenting?
  • His son has been found to have hearing loss. Is Meniere's disease typically inherited? What hereditary conditions should be considered in a young adult with sensorineural hearing loss — name two, and state the inheritance pattern and the key genetic locus for the commonest autosomal recessive non-syndromic SNHL.
  • Draft a brief, ethically sound communication to Professor Nair about the driving concern — using shared decision-making rather than a directive approach. What statutory obligations (if any) does the treating doctor have in India regarding fitness to drive in episodic vestibular disorders?