Hyperbaric oxygen as salvage treatment for idiopathic sudden sensorineural hearing loss.

OBJECTIVE: To investigate the efficacy of hyperbaric oxygen therapy as a salvage treatment for patients with sudden sensorineural hearing loss (SSHL). MATERIALS AND METHODS: Fifteen patients affected by sudden hearing loss and treated with hyperbaric oxygen after steroid and vasodilator therapy failed constituted the study group. A control group of 30 patients who were treated with steroids and vasodilator alone was also included. Hyperbaric oxygen was administered once daily for 15 sessions at a constant pressure of 2.2 atmospheres. Pure-tone hearing thresholds were obtained for both groups before and after each treatment and frequency-specific thresholds 3 months after the end of hyperbaric oxygen therapy. RESULTS: Salvage hyperbaric oxygen therapy was performed with a mean delay of 24 days from the onset of SSHL. The overall rate of hearing improvement was higher in the study group (7/15 cases, 46.6%) compared with the control group (4/30 cases, 13.3%). The mean pure-tone hearing average thresholds after salvage treatment in the study group was 53.4 dB HL (mean gain, 12.1 dB HL), which was significantly lower than that in the control group of 67.9 dB HL (mean gain, 2.7 dB HL). Hearing recovery was found to be better at low frequencies (250-500 Hz). CONCLUSION: Hyperbaric oxygen therapy should be suggested to all patients for whom initial conventional medical treatment for SSHL has failed.

Perilymphatic fistula of the round window after whiplash injury: another cause of inner ear conductive hearing loss.

Perilymphatic fistula is usually associated with sudden or fluctuating sensorineural hearing loss. We present a case of whiplash injury in a patient who showed conductive hearing loss at low frequencies due to a perilymphatic fistula occurring in the round window. Although no middle ear pathology was found, the symptoms and laboratory findings were mimicking the so called "third mobile window" phenomenon, but without the presence of inner ear dehiscence. Following early surgical exploration with patching of the round window the hearing was restored and the patient was free of symptoms.

Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype.

Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.

Benign paroxysmal positional vertigo in the first acute attack of Ménière's disease.

A 69-year-old woman, with no history of vertigo attacks, presented with the classical triad of symptoms for Ménière's disease in the right ear (vertigo, tinnitus, fullness). Upon admission, the patient had a third-degree horizontal nystagmus beating to the right, after beating towards the left ear a few hours earlier. Audiometry confirmed a severe sensorineural hearing loss in the right ear, especially at low and high frequencies. The following day, the patient complained of short episodes of vertigo linked to head movement, and the Hallpike test was compatible with benign paroxysmal positional vertigo (BPPV) in the right ear. To our knowledge this is the first description of BPPV of the posterior semicircular canal manifesting during the first vertiginous attack of Ménière's disease in the same ear. It was possible that the hydropic distension or rupture damaged the otolithic apparatus, leading to the release of otoconia debris which migrated to the posterior semicircular canal where it resulted in BPPV.

An alternative manoeuvre for posterior canal BPPV treatment.

OBJECTIVES: The aim of this study is to evaluate the effectiveness of a new manoeuvre in the treatment of posterior canal benign paroxysmal positional vertigo (p-BPPV) based on the idea that highly accelerated endolymphatic flow may lead a mass of otoconia to collide with the walls of the posterior semicircular canal, resulting in its disintegration and/or in the expulsion of the free particles from the posterior semicircular canal. MATERIAL-METHODS: Our study group included 146 patients with a diagnosis of p-BPPV. All patients underwent the new manoeuvre, which consisted of several high-acceleration successive head movements in the horizontal plane performed by the same physician. The results of the study group were compared with those of a sham control group of 30 patients with p-BPPV undergoing placebo treatment. The patients of both groups were reviewed in a follow-up appointment 1 month and 1 year after the initial treatment. RESULTS: Complete resolution of symptoms immediately after the manoeuvre was observed in 92% of patients. At 1-month and 1-year follow-up assessment, all the patients in the study group reported complete relief from their symptoms compared with only 13% and 43% of control patients respectively. Recurrence of symptoms was reported in 12 patients (8%) from the study group, who responded successfully to one additional session. CONCLUSIONS: This study establishes the efficacy of the new manoeuvre in the short- and long-term management of p-BPPV. It is a quick office procedure, usually resolving this disorder with a single session, although there some limitations in patients with underlying cervical spine pathology.

Castleman's disease of the neck: report of a case and review of the literature.

OBJECTIVES: Castleman's disease is an uncommon disease of benign lymph node hyperplasia primarily affecting the mediastinum, with the head and neck region being the second most common site. CASE REPORT: A 27-year-old woman was admitted to our department due to a left lateral cervical mass. After a complete clinical and imaging examination, a neck dissection was performed, in which a mass, size 3.5 x 2.7 x 4.5 cm, was excised. The patient was diagnosed with Castleman's disease of the neck after histopathological examination. CONCLUSIONS: The diagnosis of Castleman's disease is always a clinical challenge, as the patient commonly presents with nonspecific signs and symptoms resembling other lymphatic diseases. Consequently, Castleman's disease should be in the differential diagnosis of congenital, inflammatory, or neoplastic cervical alterations. A review of literature, including histopathological characteristics, differential diagnosis, and treatment options is also presented.

Spontaneous haematoma of the pharynx due to a rare drug interaction.

Spontaneous haemorrhage is a well known complication of oral anticoagulation therapy. Various sites of bleeding have been reported in the literature, most commonly being the gastrointestinal tract, genitourinary system and central nervous system. Spontaneous haematoma is rarely reported to involve the upper aerodigestive tract, being potentially a life threatening condition. We report a case of a 67 year-old female patient who developed spontaneous hematoma of the upper aerodigestive tract as a consequence of a rare interaction between acenocoumarol and clindamycin. Reversal of anticoagulation, careful observation, and when necessary prompt intervention to secure the airway are the main aspects of management. We highlight the need for a thorough evaluation of patients who develop new painful or other symptoms while being on anticoagulation therapy. Clinicians should be highly alerted as these could potentially be manifestations of haemorrhagic complications.

Ossicular necrosis following head injury.

PROBLEMS/OBJECTIVES: The aim of this study is to report on ossicular necrosis in the middle ear occurring shortly after head injury. METHODOLOGY: Our sample included 3 males and 2 females aged 9 to 37 years who complained of unilateral hearing loss after a head trauma that had occurred 3 to 6 months previously. The tympanic membranes were intact, and a CT-scan did not show fracture of the temporal bone. Audiometry showed a unilateral conductive hearing loss in all cases. RESULTS: On tympanotomy exploration, 4 of the 5 patients had a necrosis of the lenticular process of the incus and one patient had a necrosis of the posterior and anterior crura of the stapes. Additionally, the incus was displaced at the incudomalleolar joint towards the promontory in two patients. The long process of the incus was surgically aligned and adjusted to the head of the stapes through interposition of either temporal fascia or a bone chip. At the 2-year-postoperative follow-up, the pure tone audiometry showed that the preoperative air-bone gap was almost closed with a mean of 11.8 dB. CONCLUSIONS: It is possible that the head injury resulted in ossicular displacement in the middle ear with disturbance of the local vascular supply. Due to the tenuous blood supply to the lenticular and long processes of the incus, this portion of the ossicular chain, including the stapes suprastructure, may become more vulnerable to an ischemic process and subsequent necrosis shortly after the head trauma.

Far-advanced otosclerosis and cochlear implantation.

PROBLEMS/OBJECTIVES: To report the radiographic and surgical findings, speech perception performance, and complications of cochlear implantation for patients who were affected by far-advanced otosclerosis. METHODOLOGY: Five patients, 2 males and 3 females, with a family history of otosclerosis and who previously underwent stapedectomy to improve hearing were included in this study. CT scans of all ears were graded according to Rotteveel's grading system. All patients underwent cochlear implantation according to standard procedures. A control group of 10 non-otosclerotic postlingual implanted adults matched for age was used. RESULTS: On CT scanning, one patient had solely fenestral disease (type 1), 3 patients had localized retrofenestral disease (type 2), and 1 had diffuse retrofenestral disease with loss of the normal architecture of the cochlea (type 3). In all otosclerotic patients, the electrode array was fully inserted. However, in two patients (type 2 and 3) a thickened otic capsule was present and required more drilling than normal. One patient (type 3) experienced postoperatively facial nerve stimulation with normal fitting parameters. Otosclerotic patients showed excellent speech perception after implantation and obtained similar results to those achieved by the non-otosclerotic patients. CONCLUSIONS: Patients suffering from far-advanced otosclerosis may benefit from cochlear implantation and achieve speech performance scores comparable to non-otosclerotic implantees. Regarding surgery and facial nerve stimulation, attention should be taken to these cases in which the extension of otosclerosis is more severe on CT scanning (type 2 and mainly 3). Postoperative facial nerve stimulation can be managed successfully by resetting the current levels for comfort level.

[Spontaneous temporomandibular joint herniation into the external auditory canal]. / Hernie spontanée de l'articulation temporomandibulaire dans le conduit auditif externe.

OBJECTIVES: We report a rare case of spontaneous temporomandibular joint herniation into the external auditory canal. MATERIAL AND METHODS: A 42-year-old woman consulted for recurrent right otalgia. RESULTS: The clinical examination revealed a white mass of the anterior wall of the bony external auditory canal, very close to the tympanic annulus, which was replaced by an invagination of the skin of the canal when the patient opened her mouth, consistent with temporomandibular joint herniation into the external auditory canal. CONCLUSION: Inflammatory, malignant, and traumatic lesions are known as potential causes of temporomandibular joint herniation into the external auditory canal. Spontaneous herniations are much rare, and to the best of our knowledge this is the 15th case reported in the literature. This anomaly results from a patent foramen of Huschke. Main symptoms consist in otalgia and tinnitus. In half of the cases, they are minor and no treatment is necessary.

Subclavian steal syndrome: neurotological manifestations.

The subclavian steal syndrome is characterized by a subclavian artery stenosis located proximal to the origin of the vertebral artery. In this case, the subclavian artery steals reverse-flow blood from the vertebrobasilar artery circulation to supply the arm during exertion, resulting in vertebrobasilar insufficiency. As the vertebrobasilar arterial system feeds both the peripheral and central auditory and vestibular systems, in subclavian steal syndrome, neurotological symptoms are expected because of the vertebrobasilar insufficiency. In this report, we describe three patients suffering from subclavian steal syndrome, who presented with isolated dizziness, recurrent vertigo, hearing loss and tinnitus. In two of the three cases, a positional nystagmus was detected, which was vertical in two. Abnormal saccades were documented in one, and the auditory brainstem responses were pathological in all three patients; the caloric response was reduced in only one case. Upon magnetic resonance imaging, ischaemic lesions were observed in two patients, in the brainstem and in the hemispheres, respectively. These findings suggest that the central auditory and vestibular system is more likely to be involved in the pathogenesis of neurotological symptoms in subclavian steal syndrome. Patiehts complaining of numbness of the upper arm and isolated neurotological symptoms should be thoroughly examined for subclavian steal syndrome. Furthermore, regular follow-up must be undertaken in order to prevent other neurological deficits in the vertebrobasilar arterial territory.

[Acute unilateral total deafness and vestibular findings after gunshot noise]. / Akute einseitige Ertaubung und pathologische Vestibularisbefunde nach Knalltrauma durch Gewehrschüsse.

BACKGROUND: Acute acoustic trauma is usually acquired during military service after exposure to impulse or blast wave noise. The typical audiometric shape is a notch centered at about 4 kHz with some recovery above this frequency. This is the first case of an immediate induced unilateral total hearing loss in a young soldier following exposure to gunfire noise. CASE REPORT: A 25-year-old right-handed army officer during military training, after realizing a series of five shots with a rifle (G3), immediately experienced on the right ear otalgia, tinnitus and severe hearing loss, without imbalance or dizziness. The pure tone audiogram revealed a cophosis on the right ear without any residual remnants. In order to estimate the extent of the labyrinth damage, a caloric test and vestibular evoked myogenic potentials (VEMPs) were performed, which were both abnormal. CONCLUSION: The possible mechanical and metabolic damage effects on the cochlea from the intense gunfire noise were discussed. As the caloric test showed directional preponderance and the VEMPs were totally abolished, it has been concluded that the saccule and to a lesser degree the posterior labyrinth have been also found affected. The importance of wearing hearing protectors such as ear plugs and ear muffs during exposure to intense noise was underlined.

Polyarteritis nodosa and cochlear implantation.

Polyarteritis nodosa is a systemic disease which affects the small to medium-sized muscular arteries. Sudden or progressive, bilateral hearing loss is a presenting otologic manifestation. To date, no case of cochlear implantation in patients with polyarteritis nodosa has been reported. The authors present a case of polyarteritis nodosa (confirmed by biopsy) in a 71-year-old man with progressive, bilateral sensorineural hearing loss who underwent cochlear implantation. A successful full insertion of the Nucleus 3G electrode array was achieved without surgical or post-operative complications. The patient immediately showed a positive subjective response and, at three month post-operative evaluation, had gained useful open-set speech perception. A review of five temporal bone cases with hearing loss and polyarteritis nodosa revealed the possibility of fibrosis and ossification in the basal turn of the cochlea, of which the surgeon should be aware prior to cochlear implantation.

[Estimating the prognosis of peripheral facial paralysis: is the minimal nerve excitability test still up to date?]. / Prognoseabschätzung der peripheren Fazialisparese: Ist der Minimal-Nerve-Excitability-Test noch zeitgemäss?

BACKGROUND: In this retrospective study, we assessed the long-term prognostic value of the minimal nerve excitability test (NET) by comparing the results it yielded with the House-Brackmann (HB) index in patients with the most common types of facial paralysis, Bell's palsy and traumatic facial palsy. PATIENTS AND METHODS: Three hundred and fifty patients aged 9-85 years (mean age 42.4 years; 156 male and 194 female), all of whom were treated initially with the same steroid therapy, entered on study. Patients in whom decompression surgery had been performed were excluded so as to avoid falsely optimistic prognoses. The 350 study patients were divided into two groups: group 1 was made up of 250 with Bell's palsy and group 2, of 100 with nonpenetrating traumatic facial palsy following temporal bone fracture. The NET was conducted repeatedly in all patients for 3 weeks from the start of day 3 of treatment, the value recorded on day 14 being used in the evaluation. For each patient, the result of the NET was recorded as 'normal', 'diminished' or 'without response' according to the difference between the two sides of the face. The final HB grading was determined after 1 year to check for the agreement between the electrical prognosis and the clinical outcome and thus the reliability of the prognosis indicated by the NET. RESULTS: The results indicate that a normal NET forecast a satisfactory outcome that could be classed as HB I-II in almost all the patients in both groups. Among patients who had no response on NET, 85% of those with Bell's palsy and 90% of those with traumatic facial palsy failed to recover nerve function. Diminished nerve excitability proved to be a sign of a relatively favourable prognosis: 74% of patients in each group recovered normal facial function. CONCLUSION: The NET is a method of investigation that is easily applied and can make a positive contribution to the assessment of prognosis in Bell's palsy and in traumatic facial palsy, reflecting the functional state of the facial nerve reliably in most of cases.

Low-frequency hearing assessment by middle latency responses in children with pervasive developmental disorder.

OBJECTIVE: In this study, we assessed middle latency responses (MLR) in children diagnosed with pervasive developmental disorder (PDD) in order to detect low-frequency auditory thresholds. METHODS: 35 children, 25 males and 10 females, aged below 5, diagnosed with PDD were referred to rule out any possible hearing deficit. Audiologic evaluation included free-field testing, tympanometry, and both MLR and auditory brainstem responses. A control group of 15 normal children matched for sex and age were used. RESULTS: In 9 out of 35 children (25%), low-frequency sensory hearing loss was revealed. The hearing loss was unilateral in seven children and bilateral in two, and varied from slight to severe. In four out of the nine cases (44%), parents had not suspected hearing loss, since these children always responded to sound. CONCLUSIONS: Among children diagnosed with PDD a significant proportion (25%) was affected by low-frequency sensory hearing loss, detected by MLR. In these children, when low-frequency hearing loss was found bilateral or severe unilateral, aural rehabilitation was initiated in order to improve their responses to auditory stimuli and their impaired communication with the environment. PDD children, even if their reaction to sound is normal, must be referred to thorough audiometric evaluation while low-frequency hearing level should always be tested.

Facial electroneurography on the contralateral side in unilateral Bell's palsy.

In this study, ten patients who exhibited severe unilateral Bell's palsy of the House-Brackmann grade V underwent facial electroneurography (ENoG) on the contralateral, healthy side. Serial ENoG was conducted in seven consecutive sessions within 6 months at a given current intensity level of stimulation. According to our results, all the patients presented a rise in the maximum compound-action potential (MCAP) amplitude on the healthy side within 20 to 45 days from the onset of the palsy and shortly after the onset of the recovery of the facial function. This was attributed to the central contralateral compensatory process, which restores balanced facial function. Based on our data, a hypothetical model is shown, which demonstrates the clinical course of the contralateral MCAP values and reflects the plasticity effect of the central nervous system after the onset of Bell's palsy.

Test-retest reliability of vestibular autorotation testing in healthy subjects.

Vestibulo-ocular reflex rotational chair testing in the high-frequency range is seldom performed because it requires specialized and powerful systems. But today a new method of sweep-frequency vestibulo-ocular reflex testing, the Vestibular Autorotation Test system (Western Systems Research, Inc., Pasadena, Calif.), based on active head movements increasing from 2 to 6 Hz, is available on the market. The goal of this study was to evaluate the test-retest variability of this test in healthy subjects. Twelve young adults (22 to 42 years old) without any history of auditory or vestibular dysfunction were included in the study. Subjects underwent five tests under standardized conditions with a 1-week interval. Each test consisted of three measurements of the gain and phase of the vestibulo-ocular reflex in the horizontal and vertical planes. Statistical analysis shows that the test-retest reliability of the Vestibular Autorotation Test is poor. Therefore this method cannot be used routinely to evaluate precise vestibulo-ocular reflex anomalies.