1/334. Minimal, progressive, and fluctuating hearing losses in children. Characteristics, identification, and management.Referring to specific types of hearing loss as "minimal" or "mild" seems to imply that their effects are equally mild or negligible. A growing body of literature, however, supports the notion that such losses can have a significant impact on the communicative and educational development of young children. Although OME is considered a common childhood ailment, mounting evidence suggests that it is not always benign and may contribute to significant educational and communicative difficulties in some young children when accompanied by conductive hearing loss. Even very mild bilateral and unilateral SNHL seems to contribute to problems in the areas of social and emotional function, educational achievement, and communication in some children. Because these hearing losses are so mild, they may not be immediately recognized as the source of such difficulties. The purpose of this report is to heighten the general pediatrician's awareness of the significance of even very mild or minimal hearing losses in children. As the gatekeepers for children's health care, pediatricians are typically the primary recipients of parental expressions of concern and the initiators of evaluations or referrals to address such.- - - - - - - - - - ranking = 1keywords = hearing (Clic here for more details about this article) |
2/334. Fibrous dysplasia of the temporal bone.Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. It is seen in the two first decades of life and is equally distributed in relation to the sex of patients. The temporal bone is involved in 18% of the cases. Fibrous dysplasia of the temporal bone is more commonly manifested in the external auditory canal (80%) and the inner and medium ear can also be involved. Sarcomatous alterations are rare. diagnosis is based on radiological images. Treatment is conservative and surgery is reserved for preserving function and preventing complications. The case of a male patient, 16 years old, who presented with progressive hearing loss over a 1-year period is reported.- - - - - - - - - - ranking = 0.125keywords = hearing (Clic here for more details about this article) |
3/334. Jugular bulb dehiscence in achondroplasia.Jugular bulb dehiscence--complete absence of a roof over the jugular bulb--is a rare malformation, probably present in <<< 1% of the general pediatric population. Of 126 children with achondroplasia evaluated in the Midwest Regional Bone Dysplasia Clinic, four and probably five, were identified as having such dehiscence (at least 3.2% of the children assessed). Identifying this increased incidence in achondroplasia is of some clinical relevance, particularly including risk of difficult to control bleeding at myringotomy. It may also present as otherwise unexplained hearing loss, tinnitus and self audible bruits in these children.- - - - - - - - - - ranking = 0.125keywords = hearing (Clic here for more details about this article) |
4/334. Cochlear and vestibular dysfunction resulting from physical exertion or environmental pressure changes.Inner ear damage due to physical exertion or environmental pressure changes has been described only recently. According to Goodhill there are two possible mechanisms of injury: the explosive and the implosive. The former is triggered by increased C.S.F. pressure transmitted to the perilymph space; the latter by increased pressure in the middle ear transmitted to the perilymph space through the two windows. Sudden hearing loss or dizziness, or both, are the main symptoms. The diagnosis will be established by the history, the audiogram, and the electronystagmogram (ENG). The treatment is still controversial. As the formation of a fistula is a real possibility, an exploratory tympanotomy is suggestive on suspected cases. Due to the variety of symptoms every case has to be assessed individually. A detailed history is of utmost importance.- - - - - - - - - - ranking = 0.125keywords = hearing (Clic here for more details about this article) |
5/334. Isolated congenital stapes suprastructure fixation.Isolated congenital anomalies of the ossicles are rare. The majority of cases of congenital conductive hearing loss secondary to middle-ear anomalies have other associated defects, such as atresia, microtia and craniofacial deformities. We present a rare case of isolated congenital stapes suprastructure fixation, where a monocrural stapes with a mobile footplate was attached to the promontory by bony synostosis. There was no stapedius muscle nor pyramidal process. The incus and malleus were of normal configuration. Mobilization of the stapes from the promontory resulted in improvement in hearing. We believe this is the first reported case of such an abnormality. A review of the world literature of isolated stapes suprastructure ankylosis and the classifications of minor congenital anomalies are discussed.- - - - - - - - - - ranking = 0.25keywords = hearing (Clic here for more details about this article) |
6/334. A case of endolymphatic sac tumor with long-term survival.A 72-year-old man developed left facial palsy at age 14 and left-sided hearing loss at age 20. At the age of 59, he presented with gait disturbance, and a large left cerebellopontine angle tumor was detected, which had markedly destroyed the pyramidal bone. The tumor was subtotally resected, but he required two more operations at the ages of 64 and 69 because of tumor regrowth. At the present time, recurrent tumor has destroyed the occipital bone and is invading the scalp. However, even though he has several cranial nerve palsies and cerebellar ataxia, he remains in stable condition and demonstrates long-term survival. The patient's surgical specimens revealed a papillary adenoma, which was recently thought to be of endolymphatic sac origin, although the origin of this kind of tumor, whether arising from the middle ear or from the endolymphatic sac, has not been established with certainty so far. In this paper, we provide further evidence that this tumor originates from the endolymphatic sac, based on anatomical, histopathological, and embryological evidence.- - - - - - - - - - ranking = 0.125keywords = hearing (Clic here for more details about this article) |
7/334. Inner ear abnormalities in Kabuki make-up syndrome: report of three cases.Three patients, a female and two males, 28, 15, and 14 years of age, with Kabuki make-up syndrome (KMS) were studied for middle and inner ear abnormalities by using CT scanning of the petrous bones. All three patients had bilateral dysplasia of the inner ear, i.e., hypodysplasia of the cochlea, vestibule, and semicircular canals (so-called Mondini dysplasia), whereas their middle ears had no abnormalities. audiometry demonstrated a sharp decrease in hearing of the high tone range, bilateral in one and unilateral in another, while the third patient was noncooperative. In view of these findings, it would be advisable to study each individual with KMS and hearing impairment for possible inner ear abnormalities.- - - - - - - - - - ranking = 0.25keywords = hearing (Clic here for more details about this article) |
8/334. A possible otological complication due to maxillary expansion in a cleft lip and palate patient.OBJECTIVE: We present a possible negative side effect of a sudden onset of secretory otitis media in a 12-year-old boy with unilateral cleft lip and palate who underwent maxillary expansion. The secretory otitis media caused a temporary hearing loss developed during the activation of the expander appliance. The possible causes for this complication are discussed. CONCLUSIONS: Clinicians should be aware of the possible association between maxillary expansion and secretory otitis media.- - - - - - - - - - ranking = 0.125keywords = hearing (Clic here for more details about this article) |
9/334. Sudden unilateral hearing loss with simultaneous ipsilateral posterior semicircular canal benign paroxysmal positional vertigo: a variant of vestibulo-cochlear neurolabyrinthitis?We describe 4 patients who all simultaneously developed a sudden total or partial unilateral sensorineural hearing loss and an unusual acute peripheral vestibulopathy in the same ear characterized by posterior semicircular canal benign paroxysmal positional vertigo with intact lateral semicircular canal function. Two patients also had ipsilateral loss of otolith function. The vertigo resolved in all 4 patients after particle-repositioning maneuvers. The findings of audiometry and vestibular tests indicated that the lesion responsible for this syndrome was probably located within the labyrinth itself rather than within the vestibulocochlear nerve and that it was more likely a viral vestibulocochlear neurolabyrinthitis than a labyrinthine infarction.- - - - - - - - - - ranking = 0.625keywords = hearing (Clic here for more details about this article) |
10/334. bone marrow transplantation corrects osteopetrosis in the carbonic anhydrase ii deficiency syndrome.carbonic anhydrase ii (CAII), found in renal tubules, brain, and osteoclasts, is critical in acid-base homeostasis and bone remodeling. Deficiency of CAII gives rise to a syndrome of osteopetrosis, renal tubular acidosis (RTA), and cerebral calcification with associated developmental delay. It is inherited in an autosomal recessive fashion and found most frequently in the mediterranean region and the middle east. We report 2 related Irish families with clinically severe CAII deficiency in whom the gene mutation has been fully elucidated. Two children, one from each family, have undergone allogeneic bone marrow transplantation because of severe progressive visual and hearing loss. The older 2 children had already developed cerebral calcification and marked visual loss at the time of diagnosis and were treated symptomatically. Post-transplantation evaluation at 2 and 3 years demonstrates histologic and radiologic resolution of their osteopetrosis with stabilization of hearing and vision. Both children remain developmentally delayed and continue to have RTA, and the older child has now developed cerebral calcification. Allogeneic bone marrow stem cell replacement cures the osteoclast component of CAII deficiency and retards the development of cerebral calcification, but it appears to have little or no effect on the renal lesions. (blood. 2001;97:1947-1950)- - - - - - - - - - ranking = 0.25keywords = hearing (Clic here for more details about this article) |
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