A new semi-implantable middle ear implant for sensorineural hearing loss: three-years follow-up in a pilot patient’s group

Objective: The aim of this article is to report on the long-term follow-up of a new semi-implantable middle ear device utilized for restoration of moderate-to-severe sensorineural hearing loss in a first series of subjects.

Methods: Three subjects, affected by sensorineural hearing loss, have undergone implantation of Maxum^® middle ear implant, via a transcanal approach. They all underwent an auditory assessment, paying particular attention on the pre- versus post-operative hearing levels under the unaided, best-fitted hearing aided and implant-aided conditions. The audiometric evaluation has been repeated 3 years after implantation and implemented by questionnaires aiming at the evaluation of the quality of life.

Results: The post-operative hearing threshold and discrimination in quiet appear to be similar or better than those provided by conventional hearing aids, with a concomitant improvement of the subjects’ quality of life. The application of the present device showed to be easy and reproducible, with no severe adverse effects recorded at the 3-years follow-up. Mild issues due to the external component were also observed, such as difficulty of keeping it continuously in place due to excess canal sweating in one subject, and a temporary loss of stability due to occurring irregularities of the external coating in another subject.

Conclusions: Long-term, preliminary data reveal that the Maxum^® device may provide equal or better functional gain and word recognition scores in quiet in patients with moderate-to-severe sensorineural hearing loss, in comparison to optimally fitted hearing aids, with a satisfactory improvement of their quality of life.     

Tinnitus and bilateral sensorineural hearing loss: ankylosing spondylitis or a side-effect of sulphasalazine treatment?

We report the case of a young male patient with ankylosing spondylitis treated with sulphasalazine who developed bilateral sensorineural hearing loss with tinnitus. Discontinuation of sulphasalazine led to complete resolution of tinnitus and an improvement in hearing loss. We discuss the features of this case and also review the relevant literature.     

Auditory brainstem responses to CE-Chirp® stimuli for normal ears and those with sensorineural hearing loss

Objective: Evaluation of the characteristic differences between click-and CE-Chirp-evoked auditory brainstem responses (ABRs) in normal hearing and sensorineural hearing loss. Design: A prospective study. Ears with normal hearing and with sensorineural hearing loss were evaluated. Pure-tone audiometry and click-and CE-Chirp evoked ABRs exams were conducted for all ears. Visual detection levels, wave-V amplitudes, and latencies of the ABRs were assessed. Study sample: Twenty-two ears with normal hearing and 22 ears with sloping type sensorineural hearing loss were examined. Results: In normal-hearing ears, mean amplitudes were larger for CE-chirps than for clicks at all intensities until 80 dB nHL, at which the amplitudes dropped off, presumably due to upward spread of excitation. In ears with sensorineural hearing loss, however the drop-off was less significant at 80 dB nHL. Comparisons with pure-tone audiometry findings revealed ABRs to CE-Chirps to correlate at 0.5, 1, 2, and 3 kHz, and to clicks at 1, 2, 3, and 4 kHz. Conclusions: The CE-Chirp has advantages over clicks for examining normal ears. However, under high-level stimulation, these advantages are no longer present. In ears with sensorineural hearing loss, the upward spread of excitation is less prominent. The CE-Chirps results correlate significantly to low frequency audiometric findings at 0.5 kHz, while clicks do not.     

Can prestin level be a biomarker for determining sensorineural hearing loss?

ObjectiveTo determine prestin levels in patients with sensorineural hearing loss and to assess whether the prestin level could be a determining factor in predicting sensorineural hearing loss.Material and methodsThe study was carried out with patients that presented to the Department of Otorhinolaryngology of F?rat University. Patients were divided into four groups of 30 subjects. Group 1: individuals aged ≥55 years with no hearing loss (control group); Group 2: individuals aged 20 to 55 years with no hearing loss (control group); Group 3: individuals aged 20 to 55 years with sensorineural hearing loss; Group 4: individuals aged ≥55 years with presbycusis.Following an audiometry examination, 5 cc blood was taken from all patients to assess serum prestin levels.ResultsPrestin levels were 445.32 pg/mL in Group 1; 452.79 pg/mL in Group 2; 123.64 pg/mL in Group 3; and 79.54 pg/mL in Group 4. No difference was found between the serum prestin levels of the younger patients with hearing loss (Group 3)] and of the patients with presbycusis (Group 4) (p=0.084). No difference was found between the serum prestin levels of the younger (Group 1) and the older (Group 2) patients with presbycusis (p=0.399). Significant differences (with higher levels in the control groups) were found in between the prestin levels of between Group 3 (the younger patients with sensorineural hearing loss) and Group 2 (younger controls), and between Group 4 (older patients with presbycusis) and Group 1 (older controls) (p<0.001 and p <0.001, respectively).ConclusionSerum prestin levels can be used as biomarkers for assessing patients with presbycusis and sensorineural hearing loss. They can also be used together with audiometry tests to predict the patient's potential level of hearing loss.     

Sudden sensorineural hearing loss – A contemporary review of management issues

Sudden sensorineural hearing loss (SSNHL) is an enigmatic entity, with obscure pathophysiology and debatable efficacy of the treatment agents used. An underlying cause is identified in only 10–15% of cases. The management of the remaining patients, classified as ‘idiopathic’, is empirical, and is conventionally with systemic steroids, vasodilator therapy, rheological agents, and antioxidants, to list a few amongst the host of the agents employed for the treatment. The availability of conflicting outcomes and lack of conclusive evidence has resulted in the propagation of consensus-based treatment protocols. In the present review, we discuss the various controversial issues and newer developments in the management of idiopathic SSNHL. The current review aims to present a narrative outlook of the updated evidence base available from PUBMED, augmented with relevant designated publications.     

Lyme disease--a reason for sudden sensorineural hearing loss and vestibular neuronitis?

BACKGROUND: Lyme disease has been described as one possible cause of sudden sensorineural hearing loss and vestibular neuronitis. The necessity of serological diagnosis and its therapeutic consequences have been discussed controversially. PATIENTS AND METHODS: 344 patients with acute sensorineural hearing loss and 66 patients with vestibular neuronitis were examined in retrospect. By means of ELISA (Enzygnost Borreliosis, Dade Behring Marburg) the specific prevalences of IgG- and IgM-antibodies against borrelia in serum were evaluated. The frequency of seroprevalences for both diseases were compared to those given in the literature. Neurootological findings of the seropositive patients were compared with those of seronegative and analysed statistically. RESULTS: 15.7 % of the patients with sudden sensorineural hearing loss had positive levels of IgG-antibodies. IgM-titers were elevated in 4.7 % of the patients. The seroprevalences for IgM and IgG were above those described by other investigators for the healthy population. Patients with positive IgM-antibodies showed more often low frequency hearing loss than IgG-positive patients. 18.2 % of the patients with neuronitis vestibularis had IgG- and 1.5 % IgM-antibodies against Borrelia. Whereas IgG occurred more often than known for the healthy population, IgM was within the limit for the healthy population. The seropositive group did not show any remarkable neurootological signs compared with the seronegative group. CONCLUSIONS: Because of the elevated seroprevalences Borrelia infections may be one possible but very rare cause of sudden sensorineural hearing loss and vestibular neuronitis. Low frequency hearing loss may be a sign for an infection with Borrelia as an etiological factor especially in combination with seropositive titers. In case of the presence of IgM-antibodies, patients may be treated with oral antibiotics (Doxycyclin, Cefuroxim). In patients with neuronitis vestibularis a neuroborreliosis should be excluded by means of lumbar puncture.     

Sudden sensorineural hearing loss: does application of glucocorticoids make sense?

BACKGROUND: Treatment of sudden sensorineural hearing loss (SSNHL) consists of administration of blood flow-promoting drugs with or without the addition of glucocorticoids. General guidelines based on scientific data do not currently exist. OBJECTIVE: To investigate the effect of glucocorticoids on the treatment of SSNHL. SETTING: Academic medical center. PATIENTS AND METHODS: We retrospectively analyzed the audiograms of 603 patients with SSNHL: 301 patients (cared for between January 1, 1986, and December 31, 1991) received intravenous blood flow-promoting drugs without glucocorticoids and 302 patients (cared for between January 1, 1992, and December 31, 1998) received intravenous blood flow-promoting drugs with glucocorticoids (intravenous +/- oral application). The age distribution of patients with SSNHL in lower, middle, and higher frequencies was similar in both groups. RESULTS: Patients with SSNHL in lower and middle frequencies (250-2000 Hz) who received glucocorticoids (prednisolone-21-hydrogen-succinate) showed significantly better recovery of hearing levels compared with those who did not receive glucocorticoids (P<.05). There was no significant difference at higher frequencies between the 2 groups. Patients with SSNHL throughout all frequencies (pancochlear hearing loss) who received glucocorticoids also had significantly better recovery of hearing levels compared with those who received blood flow-promoting drugs alone (P<.05). Also, patients with elevated blood sedimentation rates had better improvement of their hearing levels after receiving glucocorticoids. CONCLUSIONS: Administration of glucocorticoids should be recommended for treatment of patients with SSNHL. In particular, patients with SSNHL in the lower and middle frequency range and pancochlear hearing loss have significantly better recovery of hearing levels.     

Pseudodominants of two recessive connexin mutations in non-syndromic sensorineural hearing loss?

BACKGROUND: Hitherto more than hundred genes and gene loci for non-syndromic or syndromic deafness have been identified. Mutations in the connexin 26 gene (GJB2) account for up to 50 % of the cases of autosomal recessive hearing loss. The genes GJB2 (Connexin 26), GJB3 (connexin 31) and GJB6 (connexin 31) are located on chromosome 13q11-12. In the inner ear up to four different connexins are expressed. Connexins appertain to a group of gap junction proteins. These proteins can oligomerize to form single-membrane channels called connexons. Each connexon is composed of six subunits, that allow communication between adjacent cells by providing a channel for diffusion of ions, metabolites and second messengers. METHOD: Each of the exons and flanking splice regions of the connexin 26, 30, and 31 genes (GJB2, GJB3, and GJB6) have been analysed by direct sequencing. RESULTS: In the involved families three heterozygous mutations could be detected in the connexin 26 (GJB2) and connexin 30 (GJB6) genes. If a combination of two of those mutations occurs, 35DeltaG with 146/147DeltaC and 35DeltaG with GJB6-D13S1830 it results in hearing loss and deafness. CONCLUSION: By evidences of a familial background of hearing loss it is reasonable to analyse the connexin genes (GJB2, GJB3 and GJB6) for mutations, additionally to a specific hearing diagnostic, in order to enhance linguistic development through hearing aid or CI-implantation at an early stage.     

Sodium enoxaparin treatment of sensorineural hearing loss: an immune-mediated response?

The authors propose the existence of a new entity of autoimmune sensorineural hearing loss on the basis of diagnostic study and treatment experience with a series of 30 patients. Immunological mechanisms play an important role in the pathogenesis and natural course of various inner-ear diseases. Patients may present clinically with symptoms resembling Ménière's disease or even with sudden deafness. Currently, no widely used standard protocol for treatment of this autoimmune sensorineural hearing loss exists. Prompted by such observations, we implemented a protocol using a particular kind of heparin--sodium enoxaparin--with a low molecular weight. Patients were randomly assigned to two groups; to those in the first group, enoxaparin was administered subcutaneously at a dose of 2,000 IU twice daily for 10 days; the patients in the second group were treated with placebo. At the beginning and at the end of the therapy period, the patients were evaluated by instrumental examinations. Specifically excluded were patients with abnormal known coagulation. On discharge, all patients treated with enoxaparin presented both a subjective and objective decrease in symptoms. No patient experienced side effects from this treatment. The results indicate that administration of sodium enoxaparin abates sensorineural hearing loss in patients with autoimmune diseases. The clinical response to therapy can confirm diagnosis.     

Can permanent sensorineural hearing loss be caused by sleeping with an ear against a train window?

A patient presented to the authors with unilateral sensorineural hearing loss after falling asleep with his ear tightly pressed against a window of a moving train. This study set out to determine whether a train could generate sound levels of sufficient intensity to cause such a hearing loss. A sound level meter was used to measure the sound levels produced at the window of a moving train. Further measurements were made with a rubber attachment on the microphone, that simulated the effect of the ear stuck to the window. The sound levels were found to be amplified by the attachment but not to levels that could cause a hearing loss over a short period. In a second experiment eight healthy volunteers all perceived an increase in sound levels when their ears were pressed against a train window. It seems unlikely that sleeping with an ear against a train window can cause hearing loss, but it cannot be ruled out.     

Is corticosteroid therapy effective for sudden-onset sensorineural hearing loss at lower frequencies?

OBJECTIVE: To assess the efficacy of corticosteroid therapy for sudden-onset sensorineural hearing loss at lower frequencies. DESIGN: Retrospective, nonrandomized, controlled study. PATIENTS AND METHODS: The recovery rate, recovery/improvement rate, and period between the start of therapy and improvement of symptoms were studied in 2 groups: the control group, consisting of 36 patients treated with adenosine triphosphate disodium, kallidinogenase, and cyanocobalamin between March 1, 2000, and January 31, 2001; and the corticosteroid-treated group, consisting of 42 patients treated with corticosteroids in addition to the previously mentioned drugs between April 1, 1997, and February 29, 2000. RESULTS: No significant difference (P =.83) was noted in the recovery rate between the control group (81%) and the corticosteroid-treated group (79%), and the recovery/improvement rate was the same for the 2 groups (83%). In addition, there was no significant difference (P =.84) for the mean +/- SD period between the start of therapy and improvement of symptoms between the control group (3.9 +/- 2.7 days) and the corticosteroid-treated group (3.7 +/- 2.1 days). CONCLUSION: Corticosteroids were not effective for sudden-onset sensorineural hearing loss at lower frequencies.     

relative contributions of spectral and temporal cues for speech recognition in patients with sensorineural hearing loss

The present study was designed to examine speech recognition in patients with sensorineural hearing loss when the temporal and spectral information in the speech signals were co-varied. Four subjects with mild to moderate sensorineural hearing loss were recruited to participate in consonant and vowel recognition tests that used speech stimuli processed through a noise-excited voeoder. The number of channels was varied between 2 and 32, which defined spectral information. The lowpass cutoff frequency of the temporal envelope extractor was varied from 1 to 512 Hz, which defined temporal information. Results indicate that performance of subjects with sensorineural heating loss varied tremendously among the subjects. For consonant recognition, patterns of relative contributions of spectral and temporal information were similar to those in normal-hearing subjects. The utility of temporal envelope information appeared to be normal in the hearing-impaired listeners. For vowel recognition, which depended predominately on spectral information, the performance plateau was achieved with numbers of channels as high as 16-24, much higher than expected, given that the frequency selectivity in patients with sensorineural hearing loss might be compromised. In order to understand the mechanisms on how hearing-impaired listeners utilize spectral and temporal cues for speech recognition, future studies that involve a large sample of patients with sensorineural hearing loss will be necessary to elucidate the relationship between frequency selectivity as well as central processing capability and speech recognition performance using vocoded signals.     

Low serum folate levels: a risk factor for sudden sensorineural hearing loss?

Objective Ischemic vascular damage of the inner ear is one of the known causes of sensorineural sudden hearing loss (SSHL). Folate is an emerging risk factor associated with an increased risk of vascular damage. The aim of this study was to investigate whether low serum folate levels are associated with SSHL.

Material and Methods Serum folate levels were determined in 43 patients with SSHL and in 24 controls.

Results Folate levels were found to be significantly lower in SSHL patients than in controls (mean difference ?1.96 ng/ml; 95% CI ?3.31, ?0.59 ng/ml; p=0.006). No significant relationship between folate levels and either sex, age, cigarette smoking, alcohol consumption or hypertension was observed, while a significant relationship was found between low folate levels and high homocysteine (HCY) levels in all 43 patients (p<0.01). The potential influence of low folate levels on hearing impairment in SSHL patients can be explained by the effects on HCY metabolism and the diminution of folate antioxidant capacity.

Conclusion Further studies are needed to elucidate whether low folate levels can be considered a risk factor for SSHL.     

Piezosurgery® for removal of symptomatic ear osteoma

Piezosurgery^® is an ultrasound instrument (24.7–29.5 kHz) that is able to cut the bone without necrosis and nonmineralized tissue damage. The aim of this work has been to determine the applicability and efficiency of the piezoelectric device in the excision of symptomatic ear osteomas. 10 patients affected by osteoma of the external auditory canal (EAC) (6 right, 4 left) were enrolled. Patients underwent excision of the EAC osteoma through a transcanal approach, with the piezoelectric device. Before and 6 months after surgery, all the patients underwent pure-tone audiometry, tympanometry, transient-evoked otoacoustic emissions, distortion product otoacoustic emissions, auditory brainstem response, and electronystamographic recording. The piezoelectric device provided excellent control without side effects on the adjacent structures of the external, middle and inner ear. The piezoelectric device is a new bony scalpel using the microvibrations at ultrasonic frequency so that soft tissue (nerve, vessel, dura mater, skin, etc.) will not be damaged even on accidental contact with the cutting tip. A feature of the piezoelectric device is its good manageability, which makes it easy for a well-trained otologic surgeon to create a straight osteotomy line: this renders the piezoelectric device suitable for bone surgery and for removal osteomas of the EAC.     

Low serum folate levels: a risk factor for sudden sensorineural hearing loss?

OBJECTIVE: Ischemic vascular damage of the inner ear is one of the known causes of sensorineural sudden hearing loss (SSHL). Folate is an emerging risk factor associated with an increased risk of vascular damage. The aim of this study was to investigate whether low serum folate levels are associated with SSHL. MATERIAL AND METHODS: Serum folate levels were determined in 43 patients with SSHL and in 24 controls. RESULTS: Folate levels were found to be significantly lower in SSHL patients than in controls (mean difference -1.96 ng/ml; 95% CI -3.31, -0.59 ng/ml; p = 0.006). No significant relationship between folate levels and either sex, age, cigarette smoking, alcohol consumption or hypertension was observed, while a significant relationship was found between low folate levels and high homocysteine (HCY) levels in all 43 patients (p < 0.01). The potential influence of low folate levels on hearing impairment in SSHL patients can be explained by the effects on HCY metabolism and the diminution of folate antioxidant capacity. CONCLUSION: Further studies are needed to elucidate whether low folate levels can be considered a risk factor for SSHL.