Background: The etiology and factors influencing the prognosis of idiopathic sensorineural hearing loss are still in focus. To determine pathogenetic relevant factors, a detailed clinical characterization of patients with idiopathic sudden sensorineural hearing loss remains to be performed.Methods: The history of 103 patients who presented with a sudden sensorineural hearing loss and received a standard treatment with prednisolone und pentoxyphylline (Stennert-Schema) has been investigated with a new questionnaire. In additon, audiological and serological investigations were analysed.Results: A partial or total recovery of hearing after therapy was reported by 57 % of patients, on average 15 dB. Half of the patients sustained at least one recurrent idiopathic sensorineural hearing loss. In single cases, fibromuscular dysplasia and hypothyroidism in autoimmunthyroidism were detected. Additionally, smoking was identified as a disease modulating factor. The age of admission to the hospital was significantly reduced in the group of smokers compared to non-smokers (p = 0.02). Non-smokers reported more episodes of sensorineural hearing loss compared to smokers (p=0.02) according to their higher age. One third of the patients reported a high level of noise exposure in private and business, which was related with the sudden sensorineural hearing loss.Conclusion: By collecting an exact medical history, in more than 50% of cases etiological and predisposing factors of sudden sensorineural hearing loss can be detected which lead to further diagnostic investigations and therapies.

Objective: This case is reported in order to demonstrate the importance of detailed clinical analysis, including evaluation of personal and family history, in the differential diagnosis of sudden sensorineural hearing loss.Case report: A 50-year-old woman presented with a sudden onset of sensorineural hearing loss in her right ear. She had experienced three previous episodes of sudden sensorineural hearing loss in her left ear, at the ages of 35, 48 and 50 years. She also reported suffering two strokes with left hemiparesis due to fibromuscular dysplasia of her right internal carotid artery. A positive family history of stroke among maternal relatives suggested autosomal dominant inheritance. The patient's personal and family history suggested a rare cause of sudden sensorineural hearing loss, for which alternative therapeutic modalities may be applicable in selected cases.Conclusions: Careful follow up of any patient with sudden sensorineural hearing loss and evaluation of their personal and family history is essential, in order to uncover evidence of rare underlying causes of sudden sensorineural hearing loss. For patients with such rare diagnoses, alternative therapy and surveillance modalities may be useful in disease management, depending on pre-existing pathology. Those patients should be managed via a multidisciplinary approach, including genetic counselling, in order to achieve the best possible outcome.