Edit concept Question Editor Create issue ticket

Thickened Earlobes-Conductive Deafness Syndrome

Escher-Hirt Syndrome


Presentation

  • The present paper describes a case of WS associated with bilateral conductive hearing loss which was not related to secretory otitis media. Hyperacusis was, moreover, present in spite of the conductive deafness.[link.springer.com]
  • A number of pathologies can present with this finding, including labyrinthitis, ischemia, and intralabyrinthine schwannomas. Vertigo is typically a prominent presenting symptom along with SNHL.[appliedradiology.com]
  • It is difficult to diagnose because the disease presents like other chronic suppurative otitis media.[emedicine.medscape.com]
  • Fifty-three loci have presently been reported. The 17 deafness forms for which the genes have been identified are presented.[ommbid.mhmedical.com]
  • Otitis media without infection presents a special problem because symptoms of pain and fever are usually not present. Therefore, weeks and even months can go by before parents suspect a problem.[asha.org]
Fever
  • Hearing loss R Relapsing Polychondritis ... floppy ear Rocky Mountain spotted fever ... hearing loss S Scarlet fever ... middle ear infection Selective IgA Deficiency ... recurring ear infections Shingles ... ear damage Stroke ... hearing problems U Upper[cureresearch.com]
  • […] dysplasia of the renal… … Medical dictionary List of diseases (E) — A list of diseases in the English wikipedia.DiseasesTOC Ea Eb* EAF * Eales disease * Ear, patella, short stature syndrome * Earlobes thickened conductive deafness * Ebola hemorrhagic fever[medicine.academic.ru]
  • At one extreme is a single short period of thin, clear, noninfected fluid without any pain or fever but with a slight decrease in hearing ability.[asha.org]
  • Acute otitis media can affect one or both ears and is associated with conductive hearing loss, ear pain and fever. Chronic otitis media is a long-standing infection in the middle ear.[deafness.org.au]
  • It may be accompanied by asthma or hay fever.[aboutface.ca]
Physician
  • Written for practicing clinicians, this volume is an excellent reference for physicians, audiologists, and other professionals working with individuals with hearing loss and their families, and can also serve as a text for clinical training programs and[books.google.com]
  • If you have a history of recurrent ear infections, your physician may insert a tube in your ear drum to help the fluid drain.[starkey.com]
  • In-Depth Information The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers.[rarediseases.info.nih.gov]
  • Make certain your physician knows about your hearing condition and concerns. An article on ototoxic medications can be seen in our Tinnitus Library.[tinnitusformula.com]
  • Will my physician refer my child for these special evaluations? As a parent, you are the best person to look for signs that suggest poor hearing.[asha.org]
Falling
  • Grommets often fall out as the eardrum heals (after around 6-12 months), and if the Glue Ear comes back another procedure under general anaesthetic to replace the grommets may be necessary, and this may happen several times.[clapa.com]
  • The wax often accumulates, dries out and falls out of the ear, carrying dirt and dust with it. This is healthy in normal amounts and also coats the skin of the ear canal and acts as a water repellant.[tinnitusformula.com]
  • You can help by adding to it. ( November 2015 ) Management [ edit ] Management falls into three modalities: surgical treatment, pharmaceutical treatment, and supportive, depending on the nature and location of the specific cause.[en.wikipedia.org]
  • Furthermore, if they did not have any deleterious effects with regard to speech and language development, they often fall into the category of treatment where we do nothing.[audiologyonline.com]
  • This occurs if the scab in the operative area falls off prematurely. Bleeding may occur in about 1-4% of patients and seems to be more frequent with age.[ccent.com]
Movement Disorder
  • disorder Trigonobrachycephaly, bulbous bifid nose, micrognathia, and abnormalities of the hands and feet Friedreich ataxia Aicardi-Goutieres syndrome type 5 Corneodermatoosseous syndrome Ankyloblepharon filiforme adnatum cleft palate Renpenning syndrome[checkrare.com]
Hypertension
  • DISCUSSION Perilymphatic hypertension or "gusher" is an important complication observed during stapedotomy or espedectomia (1).[arquivosdeorl.org.br]
  • ETIOLOGIES: Left-Sided: hypertension, aortic stenosis, angina pectoris. Right-Sided: pulmonary hypertension, pulmonic stenosis. Tennessee: S4, S1, S2 sounds together have this approximate rhythm.[kumc.edu]
  • […] deficiency type 2 Enolase deficiency type 3 Enolase deficiency type 4 Enolase deficiency Enterobiasis Enteropathica Enterovirus antenatal infection Envenomization by bothrops lanceolatus Envenomization by the Martinique lancehead viper Environment associated hypertension[bioreference.net]
  • Environment associated hypertension[?] Eosinophilia-myalgia syndrome Eosinophilic cryptitis[?] Eosinophilic cystitis[?] Eosinophilic fasciitis[?] Eosinophilic gastroenteritis[?] Eosinophilic granuloma[?] Eosinophilic lymphogranuloma[?][encyclopedia.kids.net.au]
Hearing Impairment
  • Hearing impairment E Ear foreign body ... ear infection Ear wax ... ear infection F Flu ...[cureresearch.com]
  • Signs and Symptoms Conductive hearing impairment ... ...[familydiagnosis.com]
  • […] of incus absent stapes head Clinical features from OMIM: 128980 Human phenotypes related to Earlobes, Thickened, with Conductive Deafness from Incudostapedial Abnormalities: 32 # Description HPO Frequency HPO Source Accession 1 congenital conductive hearing[malacards.org]
  • However, there are many syndromes that have both vision and hearing impairment as part of the conditions that define the syndrome.[tsbvi.edu]
  • Absence of emissions, on the other hand, indicate hearing impairment. The test is done with the infant sitting on the other's lap.[ccent.com]
Hyperacusis
  • Hyperacusis was, moreover, present in spite of the conductive deafness. Surgical or prosthetic treatment of hearing loss was delayed because of hyperacusis.[link.springer.com]
  • Learn More About Topic css id: Hyperacusis Hyperacusis, or sensitive hearing, describes a problem in the way the brain’s central auditory processing center perceives noise, often leading to pain and discomfort.[entnet.org]
  • Hearing impairment W Wegener's granulomatosis ... chronic ear problems, hearing loss Williams Syndrome ... hyperacusis (sensitive hearing) X X-Linked Agammaglobulinemia ... inner ear infections Conditions listing complications: Ear symptoms: The following[cureresearch.com]
Cleft Lip and-or Palate
  • lip/cleft palate Ectropion inferior cleft lip and or palate Edinburgh malformation syndrome Edwards Patton Dilly syndrome Edwards syndrome Ee-Eh Eec syndrome without cleft lip palate Eec syndrome Eem syndrome Egg Hypersensitivity Egg shaped pupils Ehlers-Danlos[bioreference.net]
  • Ectrodactyly-ectodermal dysplasia-cleft lip/cleft palate[?] Ectropion inferior cleft lip and or palate[?] Edinburgh malformation syndrome[?] Edwards Patton Dilly syndrome[?] Edwards syndrome[?] Eec syndrome without cleft lip palate[?][encyclopedia.kids.net.au]
  • lip palate psychomotor retardation Pillay syndrome Glucose-galactose malabsorption Spastic ataxia Charlevoix-Saguenay type Autosomal dominant palmoplantar keratoderma and congenital alopecia McKusick Kaufman syndrome Branchial arch syndrome X-linked[checkrare.com]
Paresis
  • PARALYSIS or WEAKNESS: Paresis is intermittent weakness. CAUSES of Paresis: Myasthenia Gravis (fatigable weakness) Hypokalemia can result in periodic paralysis.[kumc.edu]
  • […] deficiency syndrome Ring chromosome 9 Aplasia cutis congenita intestinal lymphangiectasia Sitosterolemia Benallegue Lacete syndrome Cutis laxa, autosomal dominant PMM2-CDG (CDG-Ia) Hereditary paraganglioma-pheochromocytoma Hereditary congenital facial paresis[checkrare.com]
Polyneuropathy
  • […] imperforate anus Spastic paraplegia 7 Spastic paraplegia 26 Trichodental syndrome Spastic paraplegia 5A Wagner syndrome Spasmodic dysphonia Al Gazali syndrome Paroxysmal exertion-induced dyskinesia KCNQ2-Related Disorders Orofaciodigital syndrome 5 Polyneuropathy[checkrare.com]

Workup

  • This article reviews the pertinent anatomy of hearing, clinical workup of acquired hearing loss, and the radiologic appearance of common entities that cause acquired hearing loss.[appliedradiology.com]

Treatment

  • Name withheld, April 2018 A massive relief to have a correct diagnosis after 10 years or more treatment. We are obviously concerned about the forthcoming treatment but grateful to finally see an end in sight.[earsandhearinguk.com]
  • The only treatment that has proved effective (supported by double-blind trials) is corticosteroid treatment-usually Prednisone or Prednisolone.[hearinglosshelp.com]
  • Treatment Treatment consists of management of the underlying problem and aggressive local debridement. Topical and systemic antifungals are used as needed.[emedicine.medscape.com]
  • As such, we want to celebrate it.” – Katie Treatment Options: Do nothing.[clapa.com]
  • Members of the Deaf community who suffer from mental health problems need culturally sensitive treatment to avoid misdiagnosis and inappropriate treatment, according to a report in the March Journal of Psychiatric Practice.[checkorphan.org]

Prognosis

  • In patients with Langerhans cell histiocytosis, unifocal disease has good prognosis and responds well to local treatment. Multifocal disease also responds well to the treatment. Disseminated disease has a bad prognosis.[emedicine.medscape.com]
  • It occurs in fulminant acute pancreatitis and carries a grave prognosis.[kumc.edu]

Etiology

  • Etiology : Autosomal dominant inheritance in some cases [6]. Pathogenesis : unknown.[sonoworld.com]
  • Among the fungi, the species of Aspergillus are considered the predominant organisms implicated in the etiology of otomycosis in tropical countries.[emedicine.medscape.com]
  • The etiology is a congenital malformation that causes abnormal communication between the perilymphatic space and the subarachnoid space.[arquivosdeorl.org.br]
  • Association of COL1A1 and otosclerosis: evidence for a shared genetic etiology with mild osteogenesis imperfecta. Am J Otol 19:604–610. McKenna MJ, Kristiansen AG, Haines J. 1996.[dizziness-and-balance.com]
  • Decreased ventricular compliance is the most common etiology of S4 sound. ETIOLOGIES: Left-Sided: hypertension, aortic stenosis, angina pectoris. Right-Sided: pulmonary hypertension, pulmonic stenosis.[kumc.edu]

Epidemiology

  • Chapters on epidemiology, embryology, non-syndromic hearing loss, and syndromic forms of hearing loss have all been updated with particular attention to the vast amount of new information on molecular mechanisms, and chapters on clinical and molecular[books.google.com]
  • From the molecular data presently available, three main epidemiologic results emerge: (a) Mutations in the gene encoding a gap junction protein, connexin 26, account for about half of the cases of prelingual isolated deafness in Caucasian populations;[ommbid.mhmedical.com]
  • HIRSUTISM PHYSICAL EXAM: Return to top CHAPTER 9: MUSCULOSKELETAL EPIDEMIOLOGY : COMMON MUSCULOSKELETAL DISEASES BY AGE: Childhood: Juvenile RA, Rheumatic Fever Young adult: Reiter's Syndrome, SLE Middle Age: Fibrositis Old Age: Osteoarthritis COMMON[kumc.edu]
Sex distribution
Age distribution

Pathophysiology

  • Each form is introduced by a brief history of how the gene was discovered, followed by the description of its clinical features and the established or putative role of the encoded protein, as well as the current hypotheses concerning the associated pathophysiological[ommbid.mhmedical.com]
  • Pathophysiology [ edit ] This section is empty.[en.wikipedia.org]
  • Pathophysiology of otosclerosis. Otol Neurotol 22:249–257. McKenna MJ, Kristiansen AG, Bartley ML, Rogus JJ, Haines JL.1998. Association of COL1A1 and otosclerosis: evidence for a shared genetic etiology with mild osteogenesis imperfecta.[dizziness-and-balance.com]

Prevention

  • IS EXOSTOSIS PREVENTABLE? Exostosis is preventable by following some basic safety precautions.[californiaearinstitute.com]
  • Any obstruction can prevent sounds from passing through the middle ear and cause hearing loss. A buildup of wax, fluid in the middle ear, or a hole in the ear drum can each cause conductive hearing loss.[starkey.com]
  • Presbycusis can be prevented but once it occurs it joins the stable of other causes of sensorineural hearing loss and becomes permanent.[tinnitusformula.com]
  • How Can I Prevent Hearing Loss? Not all cases of hearing loss are preventable.[healthline.com]
  • Learn how to stay ENT healthy, prevent problems, and manage existing conditions to improve your, or a loved one’s, daily life.[entnet.org]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!