Patients with autoimmune inner-ear disease (AIED) are treated with high doses of steroids in the short term when suffering an acute hearing loss. As a consequence, substances such as methotrexate have been employed in the role of steroid-sparing agents. Additionally, it is known that tumour necrosis factor α (TNFα) is an important mediator of the inflammatory process, inhibition of which may be of benefit in AIED. This case report illustrates the use of a TNFα inhibitor in combination with methotrexate, which is known to be an effective combination in rheumatoid arthritis but has yet to be described for sensorineural hearing loss. We conclude that progressive AIED may respond well to TNFα inhibition, whilst more difficult cases, such as this example, could benefit from combining such therapy with methotrexate.

This prospective study involved 79 homozygote and heterozygote sickle cell anaemia patients (16 to 50 years old) and a control group of 40 people.

All patients underwent ENT, audiological and brainstem auditory evoked responses (BSER) examinations in order to evaluate the incidence of sensorineural hearing loss (SNHL), to identify the changes at the level of the cochlear nerve and the central pathways, and to determine the most vulnerable group, in order to intervene with early prevention and rehabilitation for this condition.

A hearing loss of greater than 20 dB at two or more frequencies was found in 36 (45.57 per cent) sickle cell patients (19 (47.22 per cent) HbSC patients and 17 (43.59 per cent) HbSS patients) and three (7.5 per cent) members of the control group. Homozygote and heterozygote patients, as well as both sexes, were equally affected.

Bilateral hearing loss occurred in 19 (52.78 per cent) patients, unilateral right-sided hearing loss in five (13.89 per cent) patients and unilateral left-sided hearing loss in 12 (33.33 per cent) patients.

Brainstem auditory evoked potential demonstrated a prolonged I–V (III–V) interpeak latency in 13 (25.35 per cent) sickle cell patients (11 men (eight with HbSS) and two women).

The hearing loss in HbSS patients was neural in nature and of earlier onset; the hearing loss in HbSC patients was usually cochlear in nature and of later onset.

Despite high medical standards and 100 per cent social security cover, the high incidence of SNHL in our sickle cell affected patients (the majority with the Benin haplotype) was probably due to their specific haematological profile and to the original geographical distribution of the disease in the tropics.

Our results highlight the necessity for early and regular hearing assessment of sickle cell patients, including BSER examination, especially in male patients with SNHL.

Electrocution is a rare cause of sensorineural deafness. We describe and discuss the first reported case in this country of domestic electrocution resulting in a bilateral sensorineural deafness.

Hearing loss associated with high-voltage electric shock is a rare entity and minimal information is available in the current literature about this condition. To our knowledge, this article represents the first case report in the literature of improvement in sensorineural hearing loss sustained due to a high-voltage electric shock.

A case report of a patient who incurred various otologic problems, including hearing loss and tinnitus, is presented. An improvement in hearing loss and tinnitus was observed in the subsequent follow up after one month. Audiological findings and possible pathophysiology of hearing loss are discussed.

We recommend that further studies be done to investigate the incidence, severity and pathophysiology of hearing loss in such cases.

Objectives: An audiometric finding of mid-frequency sensorineural hearing loss (MFSNHL), or a U-shaped pattern, is uncommon. The objective of this study is to investigate the aetiology and prognostic significance of MFSNHL.

Design: Tertiary academic referral centre-based retrospective case review and review of audiograms to determine the prevalence of this audiometric finding.

Methods: Patients with a pure tone threshold average at 1, 2, and 4 kHz at least 10 dB greater than the average at 0.5 and 8 kHz were included in this study; 35 patients met these criteria. The mean age of the patients was 34.6 years old (range 4–71 years). Twelve patients (33 per cent) were under 18 years of age. Serial audiograms were obtained for 14 patients. The notes were reviewed for any pertinent otologic history, subsequent diagnoses, management and disease course.

Results: The prevalence of MFSNHL in this practice setting is less than 1 per cent. The average hearing threshold in the mid-frequencies was 44 dB, which was 17 dB and 20 dB lower than at 0.5 Hz and 8 kHz, respectively. The pure tone average (0.5, 1, 2 kHz) was 40 dB. Sixteen patients (44 per cent) required amplification. Of all patients, 22 had hereditary hearing loss, eight had idiopathic hearing loss, and five adults had vestibular schwannomas.

Conclusions: MFSNHL is an infrequent audiometric finding. The great majority of these cases are of presumed hereditary or idiopathic aetiology, although 22 per cent of adults had vestibular schwannomas. This series presents the causes and prognosis of this audiometric pattern.

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an autosomal dominant angiopathy characterized by recurrent cerebrovascular events, migraine and dementia. We describe a case of sensorineural hearing loss as the presenting feature of this condition. We have found no previous reports in the world literature of CADASIL presenting with a sudden sensorineural hearing loss. The significance of questioning a patient with regard to family history is exemplified in this case.

This study investigated the role of sudden sensorineural hearing loss (SSNHL) as a symptom in oral anticoagulant therapy with vitamin K antagonists (Phenprocoumon; Marcumar®, Falithrom®). Vascular compromise of the cochlea due to thrombosis, embolus, reduced blood flow or vasospasm is one of the four possible pathways that can lead to SSNHL. Oral anticoagulant therapy should prevent thrombosis; if it does not the question arises as to whether the anticoagulation is working, or the wrong hypothesis of vascular compromise has been made. Patients with SSNHL during oral anticoagulant therapy who were admitted tothe ENT Department of the University Hospital in Jena from 1998 to 2001 were included. The pure-tone audiograms and the prothrombin time (PT) values before and after the event of the SSNHL were evaluated. The study found 10 patients with SSNHL during oral anticoagulant therapy. Although the audiograms showed some improvement in the majority of cases, three cases showed almost no improvement in hearing. On admission, half of the patients showed a PT-value higher than 30 per cent and in nine cases a PT-value >30 per cent could be demonstrated at least once duringtesting. It was not possible to demonstrate a relationship between the SSNHL and oral anticoagulation. Vascular compromise cannot be excluded as a cause for sudden hearing loss in patients undergoing oral anticoagulant therapy. It is possible that oral anticoagulants influence the viscosity of the plasma leading to interference with the microcirculation in the inner ear. Further research into this area is currently being conducted.

Modiolar deficiency has been reported as representing the mildest form of cochlear dysplasia. In this study the authors aimed to determine baseline values of the mid-modiolar area and modiolus volume in normal subjects. In this way they aimed to improve the detection of previously underdiagnosed subtle cochlear dysplasia in patients with sensorineural hearing loss.

Ear drops containing neomycin only rarely cause ototoxicity. The authors report on three patients with a tympanic membrane perforation who developed severe ototoxicity after use of eardrops containing 0.35 per cent neomycin. Mitochondrial DNA analysis revealed that there was no A1555G point mutation in these patients. This finding indicates that application of low concentration neomycin to the middle ear can cause severe inner ear damage even in humans who are not hyper-susceptible to aminoglycosides.

This paper reports an unusual case in which aseptic meningitis presented with sudden sensorineural hearing loss (SSNHL) associated with intralabyrinthine haemorrhage (ILH). A seven-year-old girl presented with sudden right-sided hearing loss with dizziness. She did not have a previous history of bleeding disorders. This child was assessed using audiograms and magnetic resonance imaging (MRI). The patient's hearing loss was irreversible. Steroid therapy was not effective. SSNHL associated with ILH can be one of the negative prognostic factors in children.

Inner ear involvement with sensorineural hearing loss (SNHL) has been reported in many autoimmune disorders including ulcerative colitis. The pathogenetic mechanism of hearing loss in ulcerative colitis is thought to be immune mediated. Diagnostic tests are being developed to identify inner ear autoantibodies, that may be the cause of such hearing loss. The only test that is currently available for clinical use is the Otoblot test. This, however, tests only for antibodies against bovine heat shock protein 70 which is only one of the many cross-reacting proteins against the inner ear in suspected immune-mediated hearing loss. The clinical response to steroid therapy is thus the mainstay in the diagnosis of immune-mediated hearing loss. This paper presents a series of patients with clinically suspected autoimmune hearing loss. Diagnostic assays for this condition are discussed along with a review of the recent advances in the pathogenesis and laboratory diagnosis of immune-mediated sensorineural hearing loss.

Exposure to carbon monoxide is a well-recognized cause of morbidity and mortality. Both acute accidental poisoning and chronic exposure are associated with a range of adverse health effects. We report two cases of carbon monoxide poisoning with the associated phenomenon of sensorineural hearing loss. Although hearing loss as a result of acute carbon monoxide exposure has previously been described, here we emphasize the need to consider chronic exposure to carbon monoxide as a potentially reversible cause of sensorineural hearing loss if diagnosed and treated early.

Recent reports indicate that the cochlear nerve may be absent in some cases of congenital sensorineural hearing loss. The aim of this prospective study was to determine the incidence of cochlear nerve anomaly in cochlear implant candidates with congenital hearing loss using magnetic resonance imaging (MRI). Twenty-seven patients with congenital profound bilateral sensorineural hearing loss who were being evaluated for the cochlear implant procedure were studied.

These patients had high-resolution computerized tomography (CT), through the petrous bone in axial sections. MRI examinations consisted of T1 and turbo spin echo (TSE) T2-weighted 3 mm axial images, and additional 3D Fourier Transform T2-weighted TSE sequences obtained on three different planes (axial, perpendicular and parallel to the internal auditory canal (IAC) i.e. oblique sagittal and coronal, respectively) for the purpose of cochlear nerve demonstration. Results showed that all of the 14 patients with normal CT of the temporal bone, had four distinct nerves in the distal part of the IAC on TSE-MRI. Thirteen patients demonstrated various bony malformations of the cochleovestibular system on CT. MRI revealed the absence of the cochleovestibular nerve in four patients where the IAC was very narrow or completely absent on CT. One patient with severe Mondini malformation who had an enlarged IAC demonstrated an isolated absent cochlear nerve.

Sudden sensorineural hearing loss that presents as the initial sign of haematological disease is very rare. Chronic myelogenous leukaemia has been implicated as a causative factor of sudden sensorineural hearing loss.

A 49-year-old male presented with unilateral sudden sensorineural hearing loss. The patient was found to have chronic myelogenous leukaemia during a work-up for his hearing loss. We present a case of a chronic myelogenous leukaemia patient whose first manifestation was sudden sensorineural hearing loss. We presume that cochlear vessel occlusion as a result of elevated blood viscosity was responsible for this patient’s hearing loss. Early onset of sudden deafness in a chronic myelogenous leukaemia patient may be due to the hyperviscosity syndrome and it may be possible to reverse hearing loss through early leukapheresis.

Ecstasy is a substance of abuse commonly associated with the dance scene and taken by many young people. A brief history of Ecstasy and its side-effects is given. A case of ototoxicity is presented, as an additional side-effect to the long list of complications caused by Ecstasy.

This is a report of a patient with small internal acoustic canal pathology presenting as a sudden-onset sensorineural hearing loss. Initially the patient received non-specific empirical medical therapy and the sudden-onset sensorineural hearing loss recovered rapidly. Regression of the hearing loss, despite no change in magnetic resonance imaging (MRI) findings after the medical treatment is documented and discussed.