Against the Odds: Idiopathic Sudden Sensorineural Hearing Loss—Results from Delayed Treatment

A 79-yr-old male was evaluated by the authors 4–6 wk following a rapid decline in perceived hearing in the right ear. Initial treatment with self-administered external ear canal irrigations and topical antibiotic prescribed by the patient's primary care physician resulted in no apparent improvement. The initial hearing evaluation revealed a mild-to-severe sloping sensorineural loss above 1000 Hz in the left ear and a moderately severe to severe sensorineural loss with a “flat” configuration in the right ear. There was normal word recognition in the left ear, with no measurable word recognition in the right ear. Initial treatment with oral steroids suggested some subjective improvement, and an intratympanic dexamethasone injection was performed. A repeat audiogram obtained approximately 1 mo postinjection showed significant improvement in pure tone thresholds and significant improvement in the word recognition score in the right ear. One week later a second intratympanic injection was completed, again, with follow-up in 1 mo. This case report reviews treatment options for idiopathic sudden sensorineural hearing loss (ISSNHL), including usual time lines for treatment and expected outcomes and the specific treatments and evaluation results for this patient for whom delayed treatment was unusually effective.

Audiologic Evaluation of Patients with Mucopolysaccharidosis at a Pediatric Hospital

Summary Introduction: Mucopolysaccharidosis are characterized by the deficit of lysosomal enzymes corrupting the glycosaminoglycans. Accumulating these metabolites causes several alterations, such as otologic alterations. Objective: To evaluate a number of patients with mucopolysaccharidosis at Pequeno Principe Hospital, emphasizing their audiologic alterations and establish a permanent evaluation protocol for these patients. Method: A backward-looking study of audiologic evaluation by using records of 18 patients diagnosed with mucopolysaccharidosis and attended at Pequeno Principe Hospital between January 2005 and November 2010. Results: 7 records were selected, out of which only one did not have a hearing loss, and most of them presented conductive or mixed hearing loss, and only one had an exclusively sensorineural loss. Six of these patients had also been submitted to at least one otorhinolaryngological procedure. Conclusion: Mucopolysaccharidosis patients require a permanent care and a multidisciplinary follow-up. The pediatric otorhinolaryngologist plays an important role when performing the otologic and audiologic evaluation and managing the airways, since almost all the patients evaluated presented some kind of hearing loss and an indication of otorhinolaryngologic procedure. The pattern determined in our service was at least an initial consultation when there is a suspicion and/or diagnosis of MPS and sequential consultations that were at least twice a year or more frequent, if necessary.

Revision stapes surgery

This paper reports on the analysis of 332 otosclerosis revision operations. The results have been evaluated with reference to the type of the procedure at primary surgery, the alleged cause of failure and the applied technical solution.The need for revision surgery was found higher after primary totalstapedectomy (3.4 per cent) than after partial stapedectomy (2.2 per cent) or stapedotomy (two per cent). The reason for revision varied according to the originally applied technique eg a migrated piston, a too short piston and a lateralized graft are almost exclusively foundafter total stapedectomies.The median hearing gain after revision of stapedotomy and partial stapedectomy was higher (20 dB and 18 dB respectively) than that after revision surgery for total stapedectomy (12 dB), but significantly lower than hearing gain after primary surgery (32 dB).Revisions yielded better results in the case of primary interventions with the use of a piston or piston- wire than in the case of primary interventions with a wire-type prosthesis. The risk for sensorineural loss (one per cent) was not higher than in primary surgery.

Large vestibular aqueduct syndrome and stapes fixation

An abnormally large vestibular aqueduct has a well recognized association with inner ear anomalies and it has been assumed previously to be a variant of a Mondini type of deformity (Shuknecht, 1980; Emmett, 1985). The sole radiological finding in some patients with progressive sensorineural loss has been a large vestibular aqueduct (Valvassori and Clemis, 1978; Valvassori, 1983), which is now accepted as a separate clinical entity, i.e. the large vestibular aqueduct syndrome (LVAS).A case is presented which is believed to be the first reported with unilateral LVAS and stapes fixation and also the first stapes gusher described in association with LVAS.