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Send Home Our method Usage examples Index Contact StatisticsWe do not evaluate or guarantee the accuracy of any content in this site. Children with hearing losses, or hard of hearing include those with hearing loss and children who are deaf (see full Glossary).
While children with hearing loss or hard of hearing may respond to different sounds, or auditory stimuli (see full Glossary), including speech, children who are deaf cannot receive sound in all or most of its forms. In British Columbia, Canada, children with hearing loss or hard of hearing and children who are deaf are eligible for early intervention and special needs educational and vocational support from birth until age 19 years (BC Ministry of Child and Family Development). Children with hearing losses or hard of hearing can only hear sounds at a certain level of loudness or intensity, and at a certain level of frequency or pitch in one or both ears.
Hearing losses can be bilateral (both ears) or unilateral (one ear). Children with a hearing loss greater than 90 decibels (dB) are considered deaf. These children need specialized education support or assistance to support their optimal development and learning.
Mixed or central hearing loss indicates that the hearing is limited because both the central or inner ear and the auditory nerve are damaged.
Many of them may be slow to learn new words, or have difficulty with their speech (unclear or changing sounds, e.g.
Children with hearing loss or hard of hearing already in kindergarten and primary school grades find it hard to learn to read and write (see full Glossary) as they cannot blend sounds together. Intervention for hearing loss may start after birth, as soon as the loss has been detected.
For infants and young children consultation with an early intervention consultant or deaf and hard of hearing consultant regarding ways to stimulate the child and possible medical check-ups and interventions is helpful. Ensuring that  teacher and peers receive training in other communication methods, such as sign language (ASL) (Fig. People with hearing loss or hard of hearing use oral or manual communication, or a combination of the two. Mode of communication varies with normally developing unilateral hearing loss children using oral communication; bilateral HL children using oral communication only or assisted by some sign language in the more significant hearing loss range unless they have cochlear implants where oral communication is stressed. Total Communication (see full Glossary) is a method of instruction for people with hearing loss or hard of hearing. To learn about hearing losses or hard of hearing in the middle childhood years, please visit the six to 12 part of this course. Cases that are virus positive also are positive for TNF-alpha, suggesting a possible link to autoimmune inner ear disease. In otosclerosis, the most commonly affected portion of the bone around the inner ear (otic capsule) is the anterior oval window (about 96%).
Disorders that can be mistaken for otosclerosis (a disease) include disorders of the inner ear bones (ossicles), and fluid within the middle ear. Examples of disorders of the inner ear bones other than otosclersosis include ossicular chain disruption and ossicular fixation. In congenital ossicular fixation, the normal articulation of the small ossicular bones does not occur. Bedside: The otologic clinician should confirm audiological testing suggesting a conductive hearing loss with bedside examination. VEMP testing can also be used to diagnose superior canal dehiscence and distinguish otosclerosis from SCD. The symptom of paracusis Willisii, where the patient perceives speech better in a noisy background, is said to be frequently present in otosclerosis as well as other causes of conductive hearing loss. Otosclerosis does not have to be treated, as there are no medications that have been shown to work, and it will progress or not independent of any treatment. Hearing aids are effective for conductive hearing loss and certainly are less risky than having ear surgery. Lictor (2013) reviewed all medical treatments and concluded that there is no medical treatment proven to work.
At the present writing, fluoride therapy is no longer a recommended primary treatment for otosclerosis (NIH consensus), because of its effect on other bones including the possibility of increasing the risk of hip fractures. Lippy and Berenholtz suggest adding Caltrate and 30 mg of risedronate (a biphophonate -- Actonel) in persons who do not respond to Florical and Vitamin D. The author's advice on stapedectomy (the author is not an otologic surgeon), is that the BAHA implanted hearing aid, as well as similar devices, seem better suited to treatment of otosclerosis and similar causes of conductive hearing loss than stapes surgery. Surgery should also not be performed in patients whose hearing loss is 70 dB or less unless their speech discrimination score is 80% or better. Riggs BL, Hodgson SF, O'Fallon WM, Chao EY, Wahner HW, Muhs JM, Cedel SL, Melton LJ 3rd.1990.
Effect of fluoride treatment on the fracture rate in postmenopausal women with osteoporosis. An eight month old male presented with developmental delay and epileptic spasms for the last 3 months.
Lissencephaly is a disorder of neuronal migration characterized by absent or abnormally wide gyri and abnormally thick cortex.
A 8-year old previously healthy boy presented with fever, vomiting, bleeding gums and later, seizures on day 14 of illness. A four-year old boy, first child born to second-degree consanguineous couple presented with developmental delay, microcephaly and recurrent seizures from age of 5 months. AGS is an autosomal recessive progressive encephalopathy associated with basal ganglia calcification, white matter abnormality, CSF pleocytosis, and elevated CSF interferon- I±. Figure 2: MRI scan of the brain showed diffuse symmetric, bilateral, white matter hyperintensities on T2-weighted images and T1 W images (Fig. Division of Pediatric neurology, All india institute of medical sciences (AIIMS), New delhi. A premorbidly normal, 8-years old girl presented with 5 days- history of deviation of angle of mouth towards right side and incomplete closure of the left eye. A 5-day old baby girl, born at term gestation to 3rd degree consanguineous couple, appropriate for gestation, was admitted with complaints of seizures, poor feeding and lethargy.

Radiological differential diagnoses include ethyl malonic aciduria, 3-methyl glutaric aciduria, beta-keto thiolase deficiency, maple syrup urine disease (MSUD) and Leigha€™s disease.
Figure 2: DWI images showing areas of diffusion restriction involving medulla, dorsal pons, cerebellar peduncles, cerebellar vermis and dentate nuclei, midbrain, bilateral putamina and white matter adjacent to central sulci and splenium of corpus callosum. A 12-year old girl presented with initial motor delay, mild intellectual disability and seizures associated with fever. L2-OH glutaric aciduria is a neurometabolic disorder caused by deficiency of an FAD-linked, membrane-bound mitochondrial enzyme, L-2-hydroxyglutarate dehydrogenase. VI ChildNeurocon, the 6th Annual conference of Association of Child Neurology will be held between 20-22 February 2015, at Hotel Hindusthan International, Kolkata, West Bengal. Hearing loss may be present at birth or it may occur at any stage throughout the individual’s life. The degree of hearing loss might vary, from slight, to mild, to moderate, to severe, and profound. Hearing loss can be the result of genetic or hereditary, pre-, peri- or post-natal factors. The Early Hearing Screening Program in BC provides important information on the advantages of newborn screening, as well as testimonials for parents. Children who are either hard of hearing or deaf generally need some form of special education services in order to receive an adequate education.
Although hearing loss is usually diagnosed in early adult life, the prevalence increases almost 7-fold with age (Niedermeyer et al, 2001). This may be mediated by increased activity of the renin-angiotension system (Imauchi et al, 2008).
There are some differences in opinion about this - -as about 100% of monozygotic twins match up with respect to otosclerosis (Fowler, 1966). The author's clinical experience suggests that this estimate may be an order of magnitude too high, as practically, otosclerosis is a rare diagnosis and getting rarer.
It can also involve the round window niche (30%), the internal auditory canal, and occasionally ossicles other than the stapes (Schuknecht and Kirchner 1974; Schucknecht, 1993). The reason for this is that mistakes can be made, and when there is a significant hearing loss, inexperienced audiologists may omit or use masking incorrectly. We have not found this to be useful and in fact find tymps to be almost universally normal (see tymps in figure towards top of this page). As mentioned above, VEMP's should be absent in conductive hearing loss, but present in sensorineural hearing loss.
The main reason for this is to exclude alternatives (such as tumors or Menieres-- otosclerosis does not protect you from other ear diseases), and also to keep in touch with the audiologist in regards to when a hearing aid might be indicated. Hearing aid technology has undergone tremendous advances since the invention of surgical treatment for otosclerosis. Monical is a preparation that is absorbed in the intestine rather than in the stomach and may cause less side effects. Note that the small end of the prosthesis is no longer adjacent to bone, and also that the entire prosthesis has rotated about 45 degrees clockwise. If the prosthesis migrates inward, then ear drum movement may directly stimulate the saccule utricle. This operation, which involves drilling out the bone of the mastoid, is rarely if ever offered. This may be due to increasing prevalence of measles immunization, catching up on the backlog of cases that existed before surgery was available, or just changes in how conductive hearing loss is treated. Devices like the BAHA have less risk of dizziness or failure, and more control over the outcome. Always test each patient at least twice before doing the surgery (because mistakes can happen). We ourselves would modify this to say that acoustic reflexes should be either absent or inverted.
Epilepsy is a major presentation of Lissencephaly syndromes and is usually drug-refractory.
Child was noted to have hypertension, thrombocytopenia, hemolytic anemia, uremia and elevated serum lactate dehydrogenase suggestive of atypical hemolytic-uremic syndrome.
The main radiological differential diagnosis includes Cockayne syndrome, congenital infections, in particular congenital cytomegalovirus infection and toxoplasmosis and pseudo-TORCH syndrome. 2) predominantly in the bilateral frontal, parietal and temporal subcortical white matter regions with paucity of white matter.
It commonly presents as mild developmental delay (motor and cognitive) that is either very slowly progressive or non-progressive, sometimes as static encephalopathy. These ranges depend on a child’s ability to hear speech in others, or how loud and intensely their own speech is produced.
See the list of syndromes and conditions, and also refer to the sections on Children with Biological Risk, Children with Established Risk Conditions and Children with Genetic Disorders and Children with Nervous System Disorders.
Renin blockers (tekturna -- Aliskiren) are available, as well as many angiotension convertase inhibitors (ACE), but to our knowledge, no one has attempted their use to block worsening of otosclerosis in pregnancy. Nuclear inclusions similar to paramyxoviral nucleocapsids have been observed in otosclerosis osteoclasts (McKenna et al, 1986; Chole and McKenna, 2001). Otosclerosis is thought to begin with otospongiosis, which is a localized softening of the normally very hard bone of the otic capsule (Shambough 1971). The malleus can be fixed at the anteior nuchal ligament, or the head can be adherent to the medial-most portion of the ear canal.
Lippy and Berenholz) suggest that this condition can be diagnosed with a flat sensorineural hearing loss, good speech discrimination, absent acoustic reflexes, and a family history. It is best to confirm the conductive hearing loss both with the tuning fork test (512 hz) -- both Weber and Rinne, and with the VEMP test.
This is the same patient as above (right) where the Right ear was operated, and the left ear unoperated, but both clearly had stapedial otosclerosis.

This being said, it is still difficult to see the logic and we ourselves have never seen a patient who has volunteered this observation.
It would seem to us, that one would need to consider both the risk of hearing progression, and balance it against the risk of adverse effects on other bones, when prescribing.
Cruise et al (2010) indicated that there is only low-quality evidence for sodium fluoride therapy being effective in otosclerosis. If it migrates outward, a fistula can arise at the oval window, from which the prosthesis was dislodged. Because it involves creating an otic capsule fistula, similar to that seen in superior canal dehiscence, it has many untoward effects.
The surgeon should be prepared, if necessary, to repair the ear drum prior to the operation, treat all infections prior to the operation, and remove exostoses at the time of the ear surgery. He had repeated seizures and encephalopathy from day 14, requiring three antiepileptic drugs. He had episodes of fever associated altered sensorium and seizures for 1-2 weeks from 5 months onwards every 2-3 months.
The cortical sulci, basal cisterns, and ventricular system are prominent, suggestive of cortical atrophy. At admission, baby was in peripheral circulatory failure, encephalopathy and respiratory distress. In late childhood or adolescence, a slowly progressive neurological deterioration may be noted in the form of pyramidal, extrapyramidal, and cerebellar signs, and intellectual decline. This would be consistent with the observation that it is of variable penetrance, as one would expect a single gene to be more consistent in behavior. Measles virus RNA has been found in the stapes of otosclerotic persons (Potocka-Baklazek et al, 2014).
On the other hand, about 40% of stapes from otosclerosis patients are negative for measles (Karosi et al, 2007), and it is also unclear why measles should affect the temporal bone, but not affect other bones in the body. To our knowledge, nobody has yet attempted to apply this finding with anti-TNF drugs such as entanercept. There appear to be three stages of otosclerosis -resorptive osteoclastic stages with signs of inflammation, followed by an osteoblastic stage involving immature bone, followed by mature bone formation. OPH is found in high concentration in the perilymph (Zehnder et al, 2005), and thus lack of OPG might be thought of as a possible explanation for the the otospongiosis part of otosclerosis. To us, this clinical picture would seem rather difficult to distinguish from any other type of sensorineural hearing loss.
VEMP's should be absent in a conductive hearing loss but present in sensorineural hearing loss.
Another possible explanation is that persons with sensorineural hearing loss have great problems hearing in noise, perhaps because of loss of outer hair cells, but this is simply not the case in conductive hearing loss. There is not much one can do about a failed stapes procedure other than take more CT scans adding on more radiation, and then do more surgery. The CT scan of brain on day 14 showed bulky, hypodense pons with normal supratentorial structures (figure 1A). Baby was treated with antibiotics, antiepileptics (Phenobarbitone and Fosphenytoin), and mechanical ventilation. As the treatments for cochlear otosclerosis vary from doing nothing to an unproven medical treatment (sodium fluoride), we would favor simply diagnosing such patients as having sensorineural hearing loss. The tone was increased in all 4 limbs with brisk deep tendon reflexes and extensor plantar responses.
The characteristic EEG patterns along with the clinical findings may help neurologists to consider the diagnosis of Lissencephaly. MRI two days later showed hyperintensities involving pons and cerebellar peduncles (figure 1B). The clinical and radiological findings in our patient, together with the CSF findings of an elevated lymphocytosis are highly suggestive of Aicardi- Goutiere’s syndrome.
This is called Schwartz sign -- it is not a reliable sign and should not be relied upon (Lippy and Berenholz, 2008). Repeat CT scan three days after MRI showed involvement of bilateral parietooccipital regions along with bulky pons (figure 1C). On examination, hypertelorism with horizontal oscillatory nystagmus, spasticity were noted.
This diagnosis should be suspected in children with relative megalencephaly, intellectual disability, mild gait disturbances, normal eyes, no evidence of peripheral neuropathy or organomegaly with no identifiable cause. The hypertension, uremia, seizures and encephalopathy improved gradually with plasmapheresis. The EEG showed diffuse cerebral dysfunction (Low amplitude indeterminate activity) with electrographic seizures and frequent brief ictal rhythmic discharges (BIRDs) arising from both hemispheres independently. MRI demonstrating leukoencephalopathy with predominant subcortical white matter involvement along with characterstic garland appearance of signal changes in the cerebellum (dentate nuclei involvement) and signal changes in bilateral globus pallidus one needs to suspect L2 hydroxy glutaric aciduria.
The EEG showed diffuse high voltage alpha-frequency activity intermingled with beta-frequency activity (Fig). Repeat CT scan six weeks later showed complete resolution of signal changes in pons and parietooccipital regions, confirming the diagnosis of PRES. The radiological differential diagnoses include Canavan disease and Kearns-Sayre syndrome, both of which almost always show brain stem involvement. The Urine organic acids by GCMS confirmed the diagnosis in the four of our cases with these classical MRI findings.

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