A 49 year old man presented with a 6 month history of new onset truncal ataxia and vertigo. Further enquiry revealed a 6 year history of erectile dysfunction, low energy levels, depression, significant weight loss and a 3 year history of sensorineuronal deafness confirmed by audiometry. His past medical history included ulcerative colitis requiring a colectomy 11 years previously. On clinical examination he was a thin and well tanned patient with relative hypotension (98/40 mmHg).
Endocrine testing revealed a testosterone 6.9 nmol/l, FSH <0.5 U/l, LH <0.5 U/l, Prolactin <40 mU/l, IGF1 5.0 nmol/l, growth hormone <0.2, free thyroxine 3.2 pmol/l and TSH 1.27 mU/l. A synacthen test showed a suboptimal response to ACTH (Cortisol 1.9 nmol/l pre ACTH, 18.6 nmol/l 30 minutes post ACTH). An insulin tolerance test for growth hormone reserve is awaited. An MRI scan of his pituitary gland was normal. After commencing hormonal replacement his mood improved, with higher energy levels, weight gain and resolution of his impotence. Interestingly, his requirement for his hearing aid diminished. He reported being able to manage with only one hearing aid, instead of two, during conversation. A lower volume was adequate for daily tasks and overall he felt that overall his deafness was less severe, concordant with an improvement on repeat audiometry.
Bircher was the first to describe the association of hearing impairment in patients suffering from a goitre, only later to be recognised by the myxedematous Committee of the Clinical Society of London in 1888 in a study of 69 hypothyroid patients, 38 of which were deaf. Since then a series of authors have provided reports of improvement in the hearing defect on correction of the hypometabolic state. This case illustrates the need for vigilance in assessing cases presenting with sensorineuronal deafness for systemic disease.
06 - 07 Nov 2006
Society for Endocrinology