Endothelial Dysfunction in Idiopathic Sudden Sensorineural Hearing Loss: A Review.

An endothelial dysfunction has been described in idiopathic sudden sensorineural hearing loss (ISSHL) patients. The purpose of our review was to: i) identify, evaluate and review recent research about cardiovascular risk factors involvement and signs of endothelial dysfunction in ISSHL; ii) implication of these discovering in clinical practice and future research. A Medline literature search was conducted to identify any study on the involvement of endothelial dysfunction in ISSHL, published in the English language in the last decade. The following MEDLINE search terms were used: sudden sensorineural hearing loss (SSHL) and endothelial dysfunction (text words). Additional studies were identified by hand searching the references of original articles and review articles. Studies were not excluded on the basis of the qualitative or quantitative definitions of SSHL, treatment regimens, or outcome measures. Data were extracted from included papers by a reviewer. Information on the patients, investigations, methods, interventions, and outcomes were systematically analyzed. Characteristics and results of all included studies were reviewed systematically. High levels of adhesion molecules, hyperhomocysteinemia and lower folate levels, unbalanced oxidative status, a lower value of flow-mediated dilatation of brachial artery and a reduced percentage of circulating endothelial progenitor cells in patients affected by ISSHL support the hypothesis that this syndrome should be considered as a microcirculation disorder based on endothelial dysfunction and drive clinicians to implement all the traditional strategies used for preventing cardiovascular events, to also reduce the likelihood of ISSHL occurrence.

Maps created using a new objective procedure (C-NRT) correlate with behavioral, loudness-balanced maps: a study in adult cochlear implant users.

In uncooperative patients, electrical compound action potential (ECAP) thresholds are reliable in predicting T-levels, but are not in determining the C-level profile. The present study aims to assess if the C-level profile can be predicted by a new objective procedure (C-NRT) which uses the amplitude growth function (AGF) and is based on the assumption that equal ECAP amplitudes elicit equal loudness percepts. This is a correlational study conducted in five tertiary care referral hospitals with 21 post-lingually deaf adult cochlear implant users. Two maps were created: a behavioral, bitonal balanced (BB) map and an objective map, in which T-levels were the same as in the BB map, and C-levels were obtained with C-NRT. C-NRT consisted of performing the AGF of nine electrodes, and of setting the current level eliciting a 100 µV ECAP amplitude as C-level in the map. AutoNRT was also measured. Main outcome measures were correlation between behavioral C-profile level, objective C-profile level, behavioral T-profile level and objective T-profile (AutoNRT) level; disyllabic word recognition scores in quiet and in noise conditions (SNR = + 10 and 0) with both maps. A strong correlation was found between behavioral and C-NRT-derived C-levels (mean per electrode correlation: R = 0.862, p < 0.001). C-NRT could predict behavioral C-levels with a greater accuracy than AutoNRT. Word recognition was significantly better with BB maps only in the quiet condition (p = 0.002). C-NRT is more accurate than AutoNRT in predicting the C-level profile in adult cochlear implant users. This finding encourages future application in uncooperative patients, especially in very young children.

Quality of life after cholesteatoma surgery: intact-canal wall tympanoplasty versus canal wall-down tympanoplasty with mastoid obliteration.

OBJECTIVES: The aim of this study was to evaluate, by means of the Chronic Ear Survey (CES), the quality of life of patients who had undergone either intact-canal wall tympanoplasty (ICWT) or canal wall-down tympanoplasty (CWDT) with mastoid obliteration. METHODS: This was a retrospective case review study performed at a tertiary referral center. Among 379 patients affected by middle ear and mastoid cholesteatoma operated on between November 2000 and December 2009, 50 patients who underwent ICWT and 50 who underwent CWDT with mastoid obliteration were randomly selected. The CES scores were analyzed for both groups. RESULTS: The mean scores on the CES were 6.5 ± 2.1 in patients who underwent CWDT and 6.9 ± 2.2 in patients treated with ICWT (t = -0.93; p > 0.05). No significant differences between the two groups were found on the activity restriction, symptom, or medical resource subscales (p > 0.05). CONCLUSIONS: The results of this study demonstrate that CWDT with mastoid obliteration resulted in a quality of life comparable with that after ICWT. Postoperative hearing loss is the most frequently reported problem for both techniques.

Children with cochlear implants: cognitive skills, adaptive behaviors, social and emotional skills.

OBJECTIVE: The aim of this study is to examine cognitive skills, adaptive behavior, social and emotional skills in deaf children with cochlear implant (CI) compared to normal hearing children. METHODS: The study included twenty children affected by profound hearing loss implanted with a CI compared to 20 healthy children matched to chronological age and gender. RESULTS: Results of this study indicated that 55% of children with CI showed a score in the normal range of nonverbal intelligence (IQ > 84), 40% in the borderline range (71 < IQ < 84) and 5% were in mild range(50 < IQ < 70). No significant differences were found after comparison with normal hearing children.Children with CI reported more abnormalities in emotional symptoms (p = .018) and peer problems(p = .037) than children with normal hearing. Age of CI was negatively correlated with IQ (p = .002),positively correlated with emotional symptoms (p = .04) and with peer problems (p = .02). CONCLUSIONS: CI has a positive effect on the lives of deaf children, especially if it is implanted in much earlier ages.

The role of hearing preservation on electrical thresholds and speech performances in cochlear implantation.

OBJECTIVE: The purpose of this study was to verify if the preservation of residual hearing after cochlear implantation in classic adult cochlear implant candidates implanted with a perimodiolar electrode correlates with the electric thresholds and with speech perception measures. STUDY DESIGN: Retrospective study. SETTING: Cochlear Implant Center at a tertiary referral University Hospital. PATIENTS: Forty-one patients with cochlear implant were involved in our study. They were subdivided into 2 groups according to the average preoperative hearing loss at the frequencies of 125 and 250 Hz (low-frequency pure tone average [LFPTA]). Group A included 21 patients with a mean LFPTA of 90 dB HL or lower; group B included 20 patients with a mean LFPTA of greater than 90 dB HL. They underwent 12 months' follow-up after cochlear implantation, including standard audiologic investigation, speech discrimination tests, and electrical measurements. RESULTS: No significant differences were found in speech perception tests among groups. In group A, hearing was preserved in 8 (group A1) and was lost in 13 patients (group A2); the postoperative LFPTA was significantly better (p < 0.0001) in A1. Group A1 patients present significantly higher C values (p < 0.0001) than the other 2 groups, and the dynamic range was therefore significantly wider (p < 0.001). CONCLUSION: Preservation of residual hearing should be attempted in all cases. In fact, although effects on the speech perception are not evident with the standard evaluation, larger electrical dynamic range can be achieved and may represent a sign of cochlear "well-being," potentially allowing a more complex electric stimulation of the nerve.

Too much of a good thing: long-term treatment with salicylate strengthens outer hair cell function but impairs auditory neural activity.

Aspirin has been extensively used in clinical settings. Its side effects on auditory function, including hearing loss and tinnitus, are considered as temporary. A recent promising finding is that chronic treatment with high-dose salicylate (the active ingredient of aspirin) for several weeks enhances expression of the outer hair cell (OHC) motor protein (prestin), resulting in strengthened OHC electromotility and enhanced distortion product otoacoustic emissions (DPOAE). To follow up on these observations, we carried out two studies, one planned study of age-related hearing loss restoration and a second unrelated study of salicylate-induced tinnitus. Rats of different strains and ages were injected with salicylate at a dose of 200 mg/kg/day for 5 days per week for 3 weeks or at higher dose levels (250-350 mg/kg/day) for 4 days per week for 2 weeks. Unexpectedly, while an enhanced or sustained DPOAE was seen, permanent reductions in the amplitude of the cochlear compound action potential (CAP) and the auditory brainstem response (ABR) were often observed after the chronic salicylate treatment. The mechanisms underlying these unexpected, permanent salicylate-induced reductions in neural activity are discussed.

Soluble intercellular adhesion molecule 1 and soluble vascular cell adhesion molecule 1 in sudden hearing loss.

HYPOTHESIS: The aim of the present study was to evaluate the concentration of soluble intercellular adhesion molecule 1 and soluble vascular cell adhesion molecule 1 in patients affected by sudden sensorineural hearing loss (SSHL). STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS: Patients affected by SSHL were evaluated. Inclusion criteria for this study were hearing loss of more than 30 dB hearing level affecting at least 3 contiguous frequencies, normal hearing on the contralateral ear, negative history of hearing loss or ear surgery in the affected ear, and magnetic resonance with gadolinium negative for VIII cranial nerve pathologic findings. INTERVENTION: Circulating levels of soluble intercellular adhesion molecule 1 and soluble vascular cell adhesion molecule (VCAM) 1 were evaluated by means of enzyme-linked immunosorbent assay. MAIN OUTCOME MEASURES: The levels of adhesion molecules in SSHL patients were compared with those of a control group. RESULTS: Intercellular adhesion molecule 1 and VCAM-1 levels in sera of patients with SSHL were significantly higher than those of the matched control subjects (p < 0.001). Statistical analysis did not show significant differences between the 2 groups in terms of the known vascular risk factors such as total and fractionated cholesterol, triglycerides, fibrinogen, erythrocyte sedimentation rate smoking, and diabetes. CONCLUSION: The results of this study show that in SSHL patients, there is an increased expression of circulating adhesion molecules confirming the existence of an endothelial dysfunction and supporting the vascular involvement in the pathogenesis of the disease. The identification of high levels of adhesion molecules and of the endothelial dysfunction open the way to selective pharmacologic treatments able to correct the activation of endothelial cells.