ROMANIAN JOURNAL OF NEUROLOGY – VOLUME XI, NO. 3, 2012 142 CASE REPORTS SUPERIOR CANAL DEHISCENCE SYNDROME – CASE REPORT Elvira Parlea 1 , Madalina Georgescu 2,3 , Romeo Calarasu 2 1 Medical Center for Diagnosis and Treatment, ENT Department, Bucharest 2 University of Medicine and Pharmacy „Carol Davila“, Bucharest 3 Institute of Phono-Audiology and Functional ENT Surgery, Bucharest Author for correspondence: Elvira Parlea, Medical Center for Diagnosis and Treatment, ENT Department, Str. Washington nr. 8-10, Bucharest e-mail: elviraparlea@yahoo.com ABSTRACT We report a case of a patient with superior semicircular canal dehiscence syndrome, a recently described condi- tion in which vestibular imbalance and/or hearing loss results from the discontinuity of the bone overlying the su- perior semicircular canals. Case report. A 46-years-old women presented with autophony in the left ear and imbalance when shouting (Tullio phenomenon). Temporal bone computer tomography revealed a defect of the left superior semicircular canal caused by an enlarged superior petrosal sinus receiving drainage from a large cerebellar developmental venous anomaly. Conclusion. We review superior semicircular canal dehiscence syndrome and its management, and we discuss common aetiologies. We conclude that superior semicircular canal dehiscence syndrome may present with a solely developmental aetiology, despite presenting late in life. Key words: semicircular canal dehiscence, vertigo, hearing loss INTRODUCTION Superior canal dehiscence syndrome (SCD syn- drome) is a rare medical condition of the inner ear, caused by a thinning or complete absence of the part of the temporal bone overlying the superior semicircular canal of the vestibular system. This may result from slow erosion of the bone or physi- cal trauma to the skull and there is evidence that the defect or susceptibility is congenital. The SCD syn- drome was first described by Dr Lloyd B. Minor in 1998. SCD syndrome can affect both hearing and balance, to different extents in different patients. CASE REPORT We present the case of a patient suffering of a sound-induced loss of balance, often associated with loud noises (Tullio phenomenon). The patient described a wide range of sounds that affect bal- ance: a knock of the door, a telephone ringing and music. A change of pressure within the middle ear, for example when flying, caughing or nose-blow- ing, may equally set off a bunt of disequilibrium, known as the Hennebert sign. The patient also claimed a progressive hearing loss in the left ear. The clinical examination started with the otomi- croscopy which reveals a normal aspect of the tym- panic membrane. Acoumetry revealed a negative Rinne in the left ear and a lateralized Weber to the left ear. Videonystagmography (VNG) revealed the ab- sence of the spontaneous nystagmus; positive pres- sure within the middle ear (Valsalva) leads to a vertical-torsional nystagmus “going down”, with the fast phase to the right ear, and vertigo. Audiological evaluation included pure tone au- diometry (PTA) which revealed mild low-frequen- cy conductive hearing loss in left ear (Fig. 1) and