Ipoacusie Sindromiche

--

L'impianto cocleare

--

L'impianto cocleare

--

Dilatazione dell'acquedotto vestibolare, del dotto e del sacco endolinfatico, aspetti neuroradiologici

--

Coupling the Vibrant Soundbridge to cochlea round window: auditory results in patients with mixed hearing loss.

OBJECTIVE: To assess the functional results of the Vibrant Soundbridge (VBS) placed on the round window (RW) in patients with mixed hearing loss. STUDY DESIGN: Retrospective evaluation of functional hearing, with measurements performed 7 to 9 months postoperatively. SETTINGS: Two tertiary referral hospital centers. SUBJECTS: Twelve individuals with mixed severe hearing loss associated with chronic suppurative otitis media and otosclerosis. INTERVENTION: Surgical placement of the VBS mechanical effector in close contact with the RW membrane to directly drive the inner ear fluids. MAIN OUTCOME MEASUREMENT: Functional hearing gain as analyzed via pure-tone audiometry and speech audiometry with VBS off and on in quiet and in noise. RESULTS: We observed a mean gain of 37.5 dB (0.5-4 kHz) with wide individual differences. The overall gain is mainly due to the air-bone gap recovery, whereas a further 12-dB mean improvement of air-conducted threshold is evident at 2 kHz. The speech reception threshold in quiet shows a mean gain of 24 dB, whereas in noise, it requires a signal-to-noise ratio 7 to 13 dB greater than normal-hearing controls. All patients are daily users of their VBS device. CONCLUSION: A middle ear implant capable of directly driving the cochlear fluids seems to be a promising alternative for individuals with a severe to profound mixed hearing loss. However, variability in hearing recovery is great, likely reflecting variability in responsiveness of the cochlea associated with chronic pathologic findings and, possibly, variability in how the VBS effector interfaces with the RW. Modifying the shape of the VBS effector can improve the mechanical coupling to the RW to better exploit the device's amplification power

Sudden bilateral hearing loss: Case report of an audiological emergency

Hypertensive encephalopathy (HE) is characterized by headache, confusion, seizures and visual disturbance and may progress to coma and death. It is an acute neurological picture caused by sudden increases of arterial pressure. Bilateral deafness is a possible symptom of HE. We describe the first case of a patient affected by a hypertensive attack associated with sudden headache and bilateral hearing loss, refractory to medical treatment, whose condition evolved into coma and rapid expiry. Sudden bilateral deafness can be a warning sign of hypertensive encephalopathy. Prompt and intensive management for HE is needed for patients with sudden bilateral deafness

Intraoperative monitoring for hearing preservation and restoration in acoustic neuroma surgery.

The present article reports on our experience with hearing preservation during 158 acoustic neuroma (AN) operations via the retrosigmoid-transmeatal (RS-TM) approach with the aid of intraoperative auditory monitoring. Several auditory monitoring methods are described. Of these, the bipolar cochlear nerve action potential (CNAP) was found to be the most helpful in preserving hearing. Of 106 patients with useful hearing preoperatively, more than 50% had useful hearing after surgery. Electrical auditory brainstem responses were useful in the placement of an auditory brain stem implant (ABI) in 4 patients with neurofibromatosis type 2 (NF2). All 4 reported speech perception benefit and use their ABIs regularly in their lives. It is our firm belief that intraoperative auditory monitoring has a pivotal role in the preservation and restoration of hearing in AN surgery

Retrosigmoid approach for auditory brainstem implant.

The present paper reports our experience with the surgical retrosigmoid-transmastoid (RS-TM) technique for implanting auditory brainstem implants (ABIs). From April 1997 to August 1998, four patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation. The subjects (three men and one women) ranged in age from 22 to 31 years. Tumour size ranged from 12 to 30 mm. A classical RS-TM approach was performed. After tumour excision, identification of landmarks (VIIth, VIIIth and IXth cranial nerves, choroid plexus) to the foramen of Luschka was carefully carried out. The choroid plexus was partially removed and the tela choroidea divided and deflected. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and placed in the correct position with the help of electrically-evoked auditory brain stem responses. Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation. Details of the results are presented. In our series, the RS-TM approach represents the elective route for ABI insertion

Coupling the Vibrant Soundbridge to cochlea round window: auditory results in patients with mixed hearing loss.

OBJECTIVE: To assess the functional results of the Vibrant Soundbridge (VBS) placed on the round window (RW) in patients with mixed hearing loss. STUDY DESIGN: Retrospective evaluation of functional hearing, with measurements performed 7 to 9 months postoperatively. SETTINGS: Two tertiary referral hospital centers. SUBJECTS: Twelve individuals with mixed severe hearing loss associated with chronic suppurative otitis media and otosclerosis. INTERVENTION: Surgical placement of the VBS mechanical effector in close contact with the RW membrane to directly drive the inner ear fluids. MAIN OUTCOME MEASUREMENT: Functional hearing gain as analyzed via pure-tone audiometry and speech audiometry with VBS off and on in quiet and in noise. RESULTS: We observed a mean gain of 37.5 dB (0.5-4 kHz) with wide individual differences. The overall gain is mainly due to the air-bone gap recovery, whereas a further 12-dB mean improvement of air-conducted threshold is evident at 2 kHz. The speech reception threshold in quiet shows a mean gain of 24 dB, whereas in noise, it requires a signal-to-noise ratio 7 to 13 dB greater than normal-hearing controls. All patients are daily users of their VBS device. CONCLUSION: A middle ear implant capable of directly driving the cochlear fluids seems to be a promising alternative for individuals with a severe to profound mixed hearing loss. However, variability in hearing recovery is great, likely reflecting variability in responsiveness of the cochlea associated with chronic pathologic findings and, possibly, variability in how the VBS effector interfaces with the RW. Modifying the shape of the VBS effector can improve the mechanical coupling to the RW to better exploit the device's amplification power

Fibrous dysplasia of the temporal bone

Fibrous dysplasia is a rare, benign disease even though the clinical picture may appear severe. It presents a progressive replacement of normal bone structures by fibrous tissue, can involve one or more bone segments and, in some cases, it could be associated with skin lesions and endocrinopathies (McCune\u2013Albright syndrome). It manifests clinically with dysmorphic syndrome, ophthalmologic or otologic signs. We report three cases of temporal polyostotic fibrous dysplasia and we review the literature on this subjec