Pseudohypacusis in children: circumstances and diagnostic strategy.

OBJECTIVES: The study attempts to specify the circumstances under which we should pay attention to children's pseudohypacusis. It evaluates the methods used to detect such cases and to determine hearing thresholds, according to the uni-or bilateralism of hearing loss. The study finally deals with the future of children diagnosed with pseudohypacusis. METHODS: The study was retrospective from January 1993 to November 2011 and prospective from December 2011 to April 2012. We included all the children between 3 and 16 years who were diagnosed with pseudohypacusis. We observed the reasons for them to consult, whether they had already been tested or had treatment, and what kind of hearing loss they displayed. All children were tested using standard pure tone audiometry and speech audiometry. Depending on the first results, other tests were conducted. They included transient evoked otoacoustic emissions (TEOEs), auditory brainstem responses (ABR) and auditory steady state responses. Families were finally contacted by phone over April 2012 in order to let them know about their child's results. RESULTS: Fifty-four children were included: 19 boys and 35 girls, with an average age of 10 year-old (±3). The simulated hearing loss (HL) was bilateral (36), unilateral (18), of perception (37), moderate HL (33), cophosis (5). Fifteen cases were linked to a family or personal history of hearing loss, while 27 cases were due to important events like adoption, abuse, verbal aggression, school problems. Before diagnosing a pseudohypacusis, 13 children had had imaging studies, 3 had been treated with corticosteroids, and 5 had hearing aids. Most of the time the presence of pseudohypacusis was suspected a discrepancy between speech reception and air-conduction pure tone thresholds, as shown by the medical test (answer on whispered voice). The diagnosis was confirmed by ABR or TEOEs, except in cases where clinic was obvious. Then family's patient and patient were reassured and informed. An audiological follow-up during either 6 months or 1 year was proposed, as well as a psychological consultation. CONCLUSION: Complementary examinations have to be performed to rule out a pseudohypacusis case before suggesting an invasive or expensive treatment (surgery or hearing aids) of children.

A case of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise.

The reasons behind sudden sensorineural hearing loss are mostly unknown, but viral infections, blood disorders, ototoxicity, noise trauma, autoimmune disease, acoustic tumor, and even mental stress may be related to the disease. In cases of hearing loss as a result of psychogenic factors, early diagnosis and adequate treatment under collaboration with the psychiatric department are crucial, since failure to take appropriate measures may result in permanent sequela. We report a case, with a review of the literature, of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise.

[N100m findings of auditory-evoked fields in patients with psychogenic hearing impairment].

We recorded the auditory-evoked fields in five patients with psychogenic hearing impairment and ten healthy controls. The maximum N100m peak amplitude and latency were measured under 1000 and 2000-Hz tone burst stimuli in each ear. The mean N100m amplitude in the patients was smaller than that in the controls. Especially, regardless of the side of stimulation, the mean amplitude in the left-hemisphere was significantly smaller in the patients than in the controls, except for the 2000-Hz stimulus condition in the left ear. There was no significant difference in the latency of the N100m peak between patients and controls. We attribute the findings of attenuation of the N100m amplitude to the peculiar auditory perception in patients with psychogenic hearing impairment. Concerning the cause of this finding, we suggest two possible mechanisms: inhibitory auditory processing and attention deficit.

Conversion deafness presenting as sudden hearing loss.

Conversion deafness is a somatoform disorder characterized by hearing loss without an anatomic or pathophysiologic lesion. Clinically, discrepancies between behavior hearing thresholds and objective electrophysiologic examinations, such as impedance audiometry, otoacoustic emissions (OAE), and auditory brainstem response (ABR), will raise the suspicion of this disorder. It is judged to be due to psychological factors and that patients do not intentionally produce the symptom. Conversion deafness is sometimes reported in children but is extremely rare among adults. Two young adults with this disease are presented. These 2 patients were both under enormous stress from the national entrance examinations for universities. Pure tone audiometry showed bilateral hearing deterioration, but OAE and ABR were normal. The hearing of both patients recovered after treatment. The diagnosis, prognosis and treatment of this disorder are also discussed. It is important to discover the psychological stress in patients with conversion deafness. This report aims to increase awareness of this condition and avoid unnecessary steroid use in its treatment.

A clinical analysis of psychogenic sudden deafness.

OBJECTIVE: Sudden deafness, in most cases, has no apparent cause. In some cases viral infection, vascular occlusion, or a cochlear membrane rupture may be responsible for the sudden loss of hearing. In this study, we analyzed etiology, audiologic, psychiatric, and clinical aspects of psychogenic sudden deafness through a retrospective chart review. Patients included were thought to have sudden deafness as a result of psychogenic causes and/or stresses. STUDY DESIGN AND SETTING: We reviewed the records of 277 patients who had been admitted to the Department of Otolaryngology, Kangbuk Samsung Hospital, from January 1997 to July 2003, with the initial diagnosis of idiopathic sudden hearing loss. RESULTS: In the medical history of 9 patients, there was an association between sudden onset of hearing loss and psychogenic events. There were 4 patients who had a preexisting mental health condition. A psychiatrist provided the classification of psychogenic sudden deafness for 7 patients (2.5%) who had organic causes ruled out. These patients showed discrepancy between pure tone audiometry and ABR. There were 2 males and 5 females; 4 patients were in their 10's, 1 in his 20's, and 2 in their 40's. Three patients had bilateral hearing loss and 4 had unilateral loss. The hearing test scores revealed: 4 patients with severe to profound hearing loss at 71 dB or higher, 2 patients with moderately severe hearing loss ranging from 56 to 70 dB, and 1 patient with moderate hearing loss from 41 to 56 dB. Hearing was restored in all patients, partially or completely, by treatment with steroid injections and psychotherapy. CONCLUSIONS: A total of 2.5% of sudden onset hearing loss cases in our study were a result of psychogenic causes. We observed distinctive features for audiologic measures, psychiatric findings, and clinical characteristics. The recovery rate and prognosis were better in patients who had accurate audiometry and were under the care of a psychiatrist. EBM RATING: C-4.

Conversion disorder in a child presenting as sudden sensorineural hearing loss.

Pseudohypoacacusis is a form of conversion disorder in which a hearing loss is found in the absence of organic disease. A case of an 11-year-old boy with auditory conversion disorder after mild head trauma is reported. The child presented with unilateral hearing loss, with no accompanying vestibular symptoms and no history of otologic disease. Auditory testing revealed severe right-sided sensorineural hearing loss, however, CT scan was normal. There was a family history of conversion disorder and the child was undergoing an emotionally stressful period. It was decided to treat the child conservatively with close monitoring and a repeat audiogram 2 weeks later. One week after presentation, the child's hearing suddenly returned to normal after a second very minor head injury. It is important to consider pseudohypoacusis or conversion disorder even in cases such as trauma, when the clinical history supports a diagnosis of hearing loss.

Hearing loss among construction workers in Edmonton, Alberta, Canada.

Hearing acuity among electricians, plumbers and pipefitters, and boilermakers with > or = 20 years of union membership was compared with that of telephone workers. Automated pure tone audiometry was performed and a questionnaire was administered. Most construction workers were exposed to occupational noise and wore hearing protection. Median thresholds for electricians and telephone workers were comparable. Thresholds of plumbers and pipefitters were higher but comparable to expected values. Boilermakers had high levels of hearing loss. Thresholds at 4000 Hz among older workers were similar for all groups and were above expected values, suggesting a cohort effect. Audiometric screening seems to be warranted for some construction workers.

Meniere's disease and endolymphatic hydrops without Meniere's symptoms: temporal bone histopathology.

We studied temporal bone histopathology in 21 ears with Meniere's disease and 24 ears with endolymphatic hydrops without Meniere's symptoms and compared the findings to those in 10 ears with presbycusis and 11 ears with normal hearing. Normal hearing ears showed less degeneration of cochlear structures than the other ears. In ears with endolymphatic hydrops without Meniere's symptoms, the degeneration of spiral ligament, hair cells, dendrites (peripheral processes) and apical spiral ganglion cells was more severe than in the other three groups. In ears with Meniere's disease and endolymphatic hydrops without Meniere's symptoms, the hair cells and dendrites were more affected than ganglion cells and there was no correlation between hair cell and ganglion cell degeneration. These findings suggest that a permanent threshold shift in late stage endolymphatic hydrops is not related to ganglion cell loss but rather to degeneration of sensory elements.

Health status of a population of infants born before 26 weeks gestation derived from routine data collected between 21 and 27 months post-delivery.

UNLABELLED: This retrospective study was designed: (a) to determine the extent to which routine data sources in the UK can provide data relating to the later health status of selected groups of infants; and (b) to use such an approach to describe the outcome of a geographically defined population of infants born before 26 weeks gestation. All infants of less than 26 weeks gestation admitted for neonatal intensive care during the period 1/1/91 and 31/12/93 whose mother's address at the time of birth was within the boundaries of the Trent Health Region were included. Health status was assessed against a previously described simple scheme and using information from existing sources only. During the 3-year period 249 infants of less than 26 weeks gestation were admitted for intensive care. Of these 66 (26.5%) survived to be discharged from the neonatal service. A further seven infants died before the age of 2 years. Of the remaining 59 four were lost to follow up (three could not be traced; one was living abroad). Of the 55 infants reviewed, 36 demonstrated no features, pre-defined in the classification scheme, of severe disability. However, only 30 children appeared to be considered entirely normal. CONCLUSION: Infants born before 26 weeks gestation and admitted for neonatal intensive care had, approximately, a 12% chance of normal survival to 2 years. A slightly smaller proportion of infants survived with significant disability. Existing routine data sources could be adapted to provide useful public health information about the outcome of 'high risk' groups of infants.

Age-related hearing loss, vitamin B-12, and folate in elderly women.

BACKGROUND: Hearing impairment is 1 of the 4 most prevalent chronic conditions in the elderly. However, the biological basis of age-related hearing loss is unknown. OBJECTIVE: The objective was to test the hypothesis that age-related hearing loss may be associated with poor vitamin B-12 and folate status. DESIGN: A thorough audiometric assessment was conducted in 55 healthy women aged 60-71 y. Hearing function was determined by the average of pure-tone air conduction thresholds at 0.5, 1, 2, and 4 kHz and was categorized into 2 groups for logistic regression analyses: normal hearing (<20 dB hearing level; n = 44) and impaired hearing (> or = 20 dB hearing level; n = 11). RESULTS: Mean age was the same (65 y) for the normal hearing and impaired hearing groups. Pure-tone averages were inversely correlated with serum vitamin B-12 (r = -0.58, P = 0.0001) and red cell folate (r = -0.37, P = 0.01). Women with impaired hearing had 38% lower serum vitamin B-12 (236 compared with 380 pmol/L, respectively, P = 0.008) and 31% lower red cell folate (425 compared with 619 nmol/L, respectively, P = 0.02) than women with normal hearing. Among participants who did not take supplements containing vitamin B-12 or folate, women with impaired hearing had 48% lower serum vitamin B-12 (156 compared with 302 pmol/L, respectively, P = 0.0007) and 43% lower red cell folate (288 compared with 502 nmol/L, respectively, P = 0.001) than women with normal hearing. CONCLUSION: Poor vitamin B-12 and folate status may be associated with age-related auditory dysfunction.

Anomalous vessel in the middle ear: the role of CT and MR angiography.

An 8-year-old child was examined because of conductive hearing loss with a retrotympanic mass on otoscopy. CT and MR angiography showed a large inferior tympanic artery traversing the hypotympanum and joining a thin, irregular internal carotid artery with a normal middle meningeal artery. These investigations, coupled with knowledge of the embryological development allowed a diagnosis of a complex vascular anomaly in the middle ear and avoided potential surgical complications.

Hearing loss related to ototoxicity in children with cancer.

Children undergoing treatment for cancer often receive agents that put them at risk for ototoxicity. Platinum-based chemotherapy, aminoglycoside antibiotics, loop diuretics, and radiotherapy are all capable of inducing inner ear damage, which may result in significant sensorineural hearing loss. Frequent audiological monitoring is necessary for the early detection of changes in hearing thresholds. Age-appropriate modification of audiological testing is essential to obtain accurate results and provide maximum comfort for pediatric patients. When hearing loss is identified promptly, consideration may be given to treatment modification and/or early intervention with hearing aids and other assistive devices. The consequences of hearing loss differ depending on the developmental stage of the child at the time that hearing loss occurs. Language acquisition may be affected in very young children, whereas educational and psychosocial concerns are paramount for the older child. The pediatric oncology nurse is instrumental in assisting the child and family who are coping with hearing loss related to cancer treatment.

Temporal bone and lateral skull base malignancy: experience and results with 81 patients.

OBJECTIVE: This study aimed to examine predictive factors and treatment outcome and to devise a staging method for malignant lateral skull base disease. STUDY DESIGN: The study design was a retrospective review of all lateral skull base surgery cases. SETTING: The study was conducted at a tertiary referral center. PATIENTS: Of 317 lateral skull base lesions, 81 patients were found to have malignant disease. Three groups were identified, and the data were analyzed according to the following: epithelial malignancies, 30 patients; malignancies of salivary gland origin, 23 patients; and malignancies of mesenchymal origin, 28 patients. The mean age was 48.5 years with a range of 5-83 years. There were 42 males and 39 females. Our mean follow-up time was 54 months with a range of 1-180 months. Forty-three patients were alive without evidence of recurrent disease, 6 were living with disease, 25 were dead of disease, 3 died of other causes, and 4 were lost to follow-up. INTERVENTIONS: All patients underwent surgery with curative intent. Forty-two received adjuvant radiation, and 8 received chemotherapy. MAIN OUTCOME MEASURES: Treatment failure, survival, cranial nerve deficits, complications, and predictive factors were analyzed. RESULTS: A 58% treatment success rate was observed. Epithelial and salivary malignancy had a poorer prognosis, and pain and facial nerve dysfunction at presentation carried a worse prognosis. CONCLUSIONS: Lateral skull base malignancy is a rare entity that continues to challenge cranial base surgeons. Cranial nerve deficits and complications are frequent. The success rate of treatment is dependent on histology and disease extent.

[Late effects of low-voltage electricity accidents. Rotator cuff tendinitis, hearing loss and neuropsychological dysfunction]. / Senfølger av lavspent strømgjennomgang. Rotatortendinose, hørselstap og mulig neuropsykologisk funksjonstap.

This article describes the symptomatology of three patients following electrical accidents. The flow of the current was from hand to hand, voltage was 220/380 V, and duration was at least a few seconds for all patients. The development of symptoms was the same, and may be explained as a thermal effect of electricity on the tissue. Initially the patients experienced transient confusion, followed by stiff muscles after 1 to 3 days, and then pain in the muscle attachments and joints close to path of the current. This pain increased slowly during the first two weeks. Recovery was gradual, but often incomplete. The case notes showed that electrical accidents may be followed by chronic rotator cuff tendinitis. The clinical examination also revealed a hearing loss at about 2,000 Hz and above 4,000 Hz. The neuropsychological testing indicated a diffuse impaired function in only one patient.

Two new otolaryngologic findings in child abuse.

We reviewed cases of early childhood hoarseness caused by vocal nodules and functional hearing loss as being possible manifestations of child abuse. This is a retrospective review set in two urban referral centers. The patients consisted of four children with vocal nodules and known histories of abuse, and four children with functional hearing loss and histories suggestive of abuse. Vocal nodules or functional hearing loss may be indicators of an abusive situation. Child abuse should be considered in the differential diagnosis of these problems.

Nonorganic hearing loss in cochlear implant candidates.

Five patients undergoing assessment for cochlear implantation have been found to have a nonorganic hearing loss (pseudohypacusis). This phenomenon has not been reported previously in this patient population. The cases are presented and the causes, detection, and management of this condition are discussed.

[Results of suboccipital removal of acoustic neurinomas in Denmark 1979-1990]. / Resultater af suboccipital acusticusneurinomfjernelse i Danmark 1979-1990.

In the period from 1979 to 1990, a series of 59 patients with 59 acoustic neuromas were operated upon in five departments of neurosurgery by at least five different neurosurgical teams, employing the suboccipital approach. The perioperative mortality rate was 8.5%. Complications including hematoma, ventricular hemorrhage, meningitis, hemiparalysis, abducens nerve paralysis, recurrent nerve paralysis, postoperative wound infection and CSF leak were observed in 21 patients (35.6%). Radical removal of the tumor was not possible in 17 patients (28.8%). Converting the postoperative facial nerve function to the House-Brackmann (HB) classification, 34 patients (57.6%) were regarded as HB VI. Reconstruction of the facial nerve was attempted in 19 patients (32.2%). Attempts at preservation of hearing were unsuccessful in all patients. Failure to attain better results and the importance of the centralized Danish model of acoustic neuroma surgery are emphasized.