Bilateral Deafness and Skin Lesions in a 23-Year-Old Man

Sumaira Nabi, MBBS; Shahzad Ahmed, MBBS; Mazhar Badshah, MBBS


October 12, 2016

Physical Examination and Workup

Figure 1

Figure 2

Figure 3

Upon clinical examination, the patient is an alert young man who is oriented to time, place, and person. His vital signs include an oral temperature of 98.6°F, a regular pulse of 70 beats/min, and a blood pressure of 120/70 mm Hg. His respiratory rate is 14 breaths/min.

His Glasgow Coma Score is 15/15, and his higher mental functions are intact. Upon general physical examination, he has subcutaneous swelling on the right side of his face and his back (Figure 1). Neurologic examination reveals visual acuity, in measurement of meters, of 6/18 on his right side and 6/36 on his left side (6/6 is equivalent to 20/20 in US customary units). Loss of the corneal reflex is noted on the left side, as well as a bilateral lower motor neuron type of facial palsy and bilateral sensorineural hearing loss.

Funduscopic examination reveals bilateral papilledema. The patient has ataxia with bilateral cerebellar signs. No signs of meningeal irritation or pyramidal weakness are observed.

The patient's abdomen is soft and nontender, without evidence of organomegaly or ascites. His bowel sounds are audible. Precordial examination reveals normal heart sounds. Auscultation of the lung fields reveals normal vesicular breathing.

On laboratory analysis, the complete blood cell count and erythrocyte sedimentation rate are within the reference range. Liver function test results, renal function test results, serum glucose levels, ECG findings, and chest radiograph are unremarkable. Nerve conduction studies are also normal.

MRI of the brain with contrast is performed and reveals bilateral, rounded, well-circumscribed masses at both cerebellopontine angles (Figures 2 and 3). These are isointense on T1-weighted imaging and hyperintense on T2-weighted imaging and fluid attenuation inversion recovery sequences, with patchy postcontrast enhancement. Obstructive hydrocephalus is also seen.


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