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Gradenigo Syndrome

Gradenigo’s syndrome is a complication of an otitis media infection and mastoiditis, involving three distinct manifestations: suppurative otitis media infection, facial pain compatible with the distribution of the trigeminal nerve and abducens nerve palsy.

Images

WIKIDATA, CC BY-SA 3.0

Presentation

Gradenigo's syndrome, otherwise referred to as Gradenigo-Lannois syndrome and petrous apicitis, primarily arises as a complication following a case of otitis media infection and inflammation of the mastoid air cells of the temporal bone, that has extended to the petrous temporal bone [1] [2]. Its typical presentation involves pain that follows the distribution of the trigeminal nerve (5th cranial nerve), alongside palsy of the abducens nerve (6th cranial nerve) and otorrhea, the latter resulting from an otitis media infection [3]. Even though the aforementioned triad is considered to be the classical presentation of Gradenigo's syndrome, clinical characteristics of the infection are often diverse; photophobia, fever, excessive secretion of tears and a diminished corneal sensitivity may further complicate the clinical picture. Patients may also present with persistent headaches and some studies even report symptomatology consistent with facial and vestibulocochlear nerve pathology [4]. If left misdiagnosed or untreated, Gradenigo's syndrome can lead to serious neurological complications and death [5].

Given that Gradenigo's syndrome occurs as a complication of an original otitis media infection that has extended to involve the apex of the petrous temporal bone, the syndrome is currently considered a rare medical entity, since antibiotics started to be used in daily clinical practice.

Entire Body System

  • Fever

    When a child presents with fever, headache and photophobia, central nervous system infections should be excluded. [bmcpediatr.biomedcentral.com]

    CASE REPORT: PHLS, 2 years, masculine sex, directed for pediatrician with picture of hyaline rhinorrhea, fever, otalgia to the right have 8 days without improvement with use of the amoxicilina. [arquivosdeorl.org.br]

    In addition, other symptoms may include: involvement of the facial nerve and vestibulocochlear nerve 5 photophobia fever excessive lacrimation Further spread of infection may give rise to complications, including: intracranial abscess meningitis skull [radiopaedia.org]

    She was without any preceding infectious symptoms including ear pain, sinus congestion, sore throat, and cough, and she denied fevers and chills. Examination demonstrated a right eye lateral gaze palsy and reproducible diplopia. [ncbi.nlm.nih.gov]

    Other principal symptoms are photphobia, excessive lacrimation, fever, and reduced corneal sensitivity. The Gradenigo triad consists of otitis, abducens paralysis, and deep pain. [whonamedit.com]

  • Malaise

    He did not have nausea, vomiting, malaise or seizures. There was no ear pain or hearing loss. He had been taking oral cefuroxime prescribed by a general practitioner for the febrile illness during previous 5 days. [bmcpediatr.biomedcentral.com]

    Fever and malaise precede neurological findings. The initial localising features are unilateral chemosis, periorbital oedema, and eyelid swelling. As retrobulbar pressure increases, ophthalmoplegia and exophthalmus ensue. [jnnp.bmj.com]

Gastrointestinal

  • Abdominal Mass

    We report a case of a non-Hodgkin's lymphoma in a young woman presenting with an abdominal mass and an unusual instance of cranial nerve palsies mimicking Gradenigo's syndrome. [ncbi.nlm.nih.gov]

    +55 11 5083 9935, E-Mail [email protected] Case Rep Neurol 2011;3:45–47 Abstract PDF Abstract We report a case of a non-Hodgkin’s lymphoma in a young woman presenting with an abdominal mass and an unusual instance of cranial nerve palsies mimicking [doi.org]

Ears

  • Otalgia

    CASE REPORT: PHLS, 2 years, masculine sex, directed for pediatrician with picture of hyaline rhinorrhea, fever, otalgia to the right have 8 days without improvement with use of the amoxicilina. [arquivosdeorl.org.br]

    A 45-years-old man initially complained of right otalgia and trigeminal neuralgia, while right abducens nerve paralysis appeared about two weeks after onset of acute otitis media. A CT scan showed an extradural abscess in the right petrous apex. [jstage.jst.go.jp]

    Full Text A male aged 41 presented with a one-month duration of otalgia and deafness in the right ear, which was later associated with binocular diplopia, fever and slight non fetid whitish discharge from the ear. [elsevier.es]

  • Tinnitus

    […] syndrome References: [8] Cerebellopontine angle syndrome Etiology : : most commonly vestibular schwannomas ( acoustic neuroma ), although other cerebellopontine angle tumors may result in the same presentation Clinical features Unilateral hearing loss Tinnitus [amboss.com]

    […] tympanic branch of the glossopharyngeal nerve (Jacobson's nerve) within the middle ear cavity Glomus jugulare - arise from paraganglia along the auricular branch of the vagus nerve (nerve of Arnold) in the jugular fossa Patients present with pulsatile tinnitus [spinwarp.ucsd.edu]

    Progressive sensorineural hearing loss is the most common presenting symptom often associated with high pitched tinnitus. Tinnitus often subsides as hearing loss progresses to complete deafness. [jnnp.bmj.com]

    A History of Tinnitus. Tinnitus handbook. San Diego, CA: Singular Thomson Learning; 2000. p. 437-48 37. Stephens D, Orzan E. Galleti Di San Cataldo F: Giuseppe Gradenigo and his contributions to audiology. J Laryngol Otol. 1997. 111: 418-23 38. [surgicalneurologyint.com]

    病期分類 シュタール耳 歩行異常 標準失語症検査 標準マイクロホン 標準聴覚閾値 定在波 アブミ骨摘出術 アブミ骨神経 同 stapedius reflex アブミ骨筋反射 " ear)reflex stapedial tinnitus アブミ骨筋性耳鳴 stapedius(muscle)tendon アブミ骨筋腱 類 acoustic(middle ! [upslide.site]

Eyes

  • Lacrimation

    In addition, other symptoms may include: involvement of the facial nerve and vestibulocochlear nerve 5 photophobia fever excessive lacrimation Further spread of infection may give rise to complications, including: intracranial abscess meningitis skull [radiopaedia.org]

    Other principal symptoms are photphobia, excessive lacrimation, fever, and reduced corneal sensitivity. The Gradenigo triad consists of otitis, abducens paralysis, and deep pain. [whonamedit.com]

    Other symptoms can include photophobia, excessive lacrimation, fever, and reduced corneal sensitivity. The syndrome is usually caused by the spread of an infection into the petrous apex of the temporal bone. [ipfs.io]

    […] syndrome include: retroorbital pain due to pain in the area supplied by the ophthalmic branch of the trigeminal nerve (fifth cranial nerve), abducens nerve palsy (sixth cranial nerve)[4] otitis media Other symptoms can include photophobia, excessive lacrimation [en.wikipedia.org]

  • Eye Pain

    He also complained of headache, left eye pain and photophobia for the same duration and double vision for 2 days. He did not have nausea, vomiting, malaise or seizures. There was no ear pain or hearing loss. [bmcpediatr.biomedcentral.com]

    Eye findings may include inflammation of the uvea (uveitis), red eyes, eye pain, eye irritation, photophobia, and vision or refractive error changes. There are several categories of JIA. [michiganorthopticprogram.com]

    […] movement eye pain eye tracking test 同 cholesteatoma of external auditory canal 外耳道真珠腫 ! [upslide.site]

  • Blepharitis

    The ocular findings in individuals with Down syndrome include refractive errors, astigmatism, strabismus, nystagmus, amblyopia, cataracts, glaucoma, Brushfield spots, blepharitis, lacrimal obstructions, and corneal ectasias. [michiganorthopticprogram.com]

    […] arginine deiminase アルテルナリア症 alternariasis アルブミン albumin Alb アルベカシン arbekacin ABK (HBK) アレルギー allergy 即時型アレルギー immediate type allergy 遅延型アレルギー delayed type allergy 光アレルギー photo allergy ペニシリンアレルギー penicillin allergy 薬物アレルギー drug allergy アレルギー性眼瞼炎 allergic blepharitis [docsplayer.net]

Face, Head & Neck

  • Facial Pain

    Inflammation in this region can damage these nerves, causing facial pain and horizontal diplopia secondary to unilateral esotropia. [eyewiki.aao.org]

    A 60-year-old woman presented to the emergency department with 7 days of right-sided headache, facial pain, and diplopia. She awoke with the headache and facial pain 7 days earlier. [ncbi.nlm.nih.gov]

    Abstract Introduction: Gradenigo’s syndrome consists of ophthalmoplegia, facial palsy, and facial pain due to involvement of abducens and facial nerves as well as the trigeminal ganglion. [jjhres.com]

Neurologic

  • Headache

    A 60-year-old woman presented to the emergency department with 7 days of right-sided headache, facial pain, and diplopia. She awoke with the headache and facial pain 7 days earlier. [ncbi.nlm.nih.gov]

    When a child presents with fever, headache and photophobia, central nervous system infections should be excluded. [bmcpediatr.biomedcentral.com]

    In as the day of antibiotic therapy after had regression of the chronic headache and the rigidity of nape of the neck, presenting complete remission of the paralysis of 6th cranial pair 10 days of antibiotic therapy. [arquivosdeorl.org.br]

    Our patient was admitted to the pediatric unit after review from a local ear nose and throat surgeon with the development of diplopia and intractable headache. [journals.lww.com]

    He received a 6-week course of antibiotics and had subjective improvement of the headache, hearing loss, diplopia, and retro-orbital pain. [acpjournals.org]

  • Trigeminal Neuralgia

    Background: Gradenigo´s Syndrome usually appears as a complication of the suppurative otitis media and its triad consists of acute otitis media, trigeminal neuralgia and sixth cranial nerve palsy. [jns-journal.com]

    A 45-years-old man initially complained of right otalgia and trigeminal neuralgia, while right abducens nerve paralysis appeared about two weeks after onset of acute otitis media. A CT scan showed an extradural abscess in the right petrous apex. [jstage.jst.go.jp]

    neuralgia and ipsilateral abducens nerve palsy. [revistas.rcaap.pt]

    Trigeminal nerve (5th CN) involvement explains Trigeminal neuralgia - pain in the distribution of the trigeminal nerve manifest with hemicranial headache, hemi facial pain. 3. [neuroradiologycases.com]

    The clinical picture is of general interest, as the pain is in the form of a trigeminal neuralgia, except that it is limited to the first division of the fifth nerve and may persist for weeks or months after the discharge from the ear has ceased. [jamanetwork.com]

  • Hyperesthesia

    Most of the patients present with hyperesthesia of the trigeminal area innervation, especially of the ophthalmic branch, diplopia and evidence of middle ear infection. [oldfiles.bjorl.org]

    ヒトパピローマウイルス ハント症候群 ハッチンソン徴候 同 respiratory distress syndrome of 新生児呼吸窮迫症候群 " newborn マダニ ヒアルロニダーゼ試験 同 cystic lymphangioma ヒグローマ,嚢胞性リンパ管腫 " 舌骨喉頭蓋靭帯 舌骨舌筋 同 acoustic hyperesthesia 聴覚過敏 " 高圧酸素療法 過敏性非感染性鼻炎 同 nasal hypersensitivity 鼻過敏症 " 多分割照射 同 hyperfunctional [upslide.site]

  • Ocular Bobbing

    O 8 6 occ O occult carcinoma occulusion effect occupational therapist octave octave band analyzer ocular bobbing ocular counter-rolling ocular deviation ocular flutter ocular following ocular tilt reaction odontogenic maxillary sinusitis off-vertical [upslide.site]

Workup

The first step towards a precise diagnosis of Gradenigo's syndrome is a complete medical history, involving symptomatology and duration thereof and a confirmation of a recent or chronic otitis media infection. Manifestations that include an abducens nerve palsy, pain along the distribution of the trigeminal nerve and an otitis media infection that also involves the discharge of pus greatly direct suspicion towards Gradenigo's syndrome.

Imaging modalities that are used to diagnose the syndrome encompass a computerized tomography scan (CT scan) and a magnetic resonance imaging scan (MRI scan) of the temporal bone. The former usually illustrates hypodensity in the region of the petrous apex; the latter is expected to display hyperintensity on T2-weighted images and hypointensity on T1-weighted images. The infected region is enhanced in both the CT and the MRI scan. An MRI scan can also exhibit the pathology that underlies Gradenigo's syndrome and can range between cholesteatomas, malignant tumors, inflammatory granulomas and osteomyelitis [6] [7]. A single study also implemented diffusion-weighted MRI to aid in the depiction of abscesses and cholesteatomas in regions where diffusion is limited [6].

The differential diagnosis of Gradenigo's syndrome also entails various other pathologies that can assume the same symptomatology. Nasopharyngeal carcinoma and lymphoma of the petrous bone are two conditions whose symptomatology can greatly resemble that of Gradenigo's syndrome. Attention should be brought to the possibility of afebrile, non-otological manifestations after infectious petrositis, which could be misdiagnosed as a malignancy, with the consequence of antimicrobial treatment postponement influencing the prognosis [8].

Treatment

CONCLUSION: Diagnosis fast e beginning of the treatment is mandatory in the cases of complicated acute average otitides. [arquivosdeorl.org.br]

General treatment High dose antibiotic therapy is the primary form of treatment. In cases where inflammation puts key structures at risk, surgery is performed, most commonly mastoidectomies and petrosectomies [9]. [eyewiki.aao.org]

Although there is little evidence to support the use of medical therapy in the treatment of Gradenigo's syndrome resulting from chronic ear disease, we here demonstrate successful conservative treatment of Gradenigo's syndrome following chronic otitis [ncbi.nlm.nih.gov]

With changing medical technology, a review of the diagnostic and treatment options for this rare but serious condition, is timely. [cambridge.org]

The patient responded to prolong antibiotic treatment for 3 weeks after which the lateral rectus palsy completely recovered. [clinmedjournals.org]

Prognosis

Treatment and prognosis The mainstay of treatment is antibiotic therapy 4. Rare cases may require surgical intervention. [radiopaedia.org]

Attention should be brought to the possibility of afebrile, non-otological manifestations after infectious petrositis, which could be misdiagnosed as a malignancy, with the consequence of antimicrobial treatment postponement influencing the prognosis. [symptoma.com]

Prognosis Formerly, the prognosis was very poor with death a common occurrence. [patient.info]

A clinical study comprising 124 cases with special reference to clinical presentation and prognosis. Neurosurg Rev 1998;21:243-8. [Figure 1], [Figure 2] [cancerjournal.net]

prevertebral or parapharyngeal abscess [8] Spread of infection to cavernous sinus [19] Spread to skull base (Vernet's syndrome) [8] Hearing loss [11][18] Epidural or subdural empyema [19] Sigmoid sinus thrombosis [19] Hydrocephalus [19] Death [13] Prognosis [eyewiki.aao.org]

Etiology

The majority of published cases involve children, and the most common etiology is otitis media. We report a case of a diabetic man who presented with repeating Gradenigo's syndrome symptoms due to NPC. [ncbi.nlm.nih.gov]

[…] to the trigeminal nerve References: [1] [2] Foster-Kennedy syndrome Orbital apex syndrome Cavernous sinus syndrome Etiology Cavernous sinus thrombosis (e.g., due to sinusitis ) Carotid-cavernous fistula Cavernous sinus tumors Carotid artery aneurysms [amboss.com]

The etiological cause for it must be found by proper imaging and accordingly it must be effectively treated with long duration antibiotic coverage. [clinmedjournals.org]

The most common etiology of this syndrome is an infection.[2] We reported a case that differs from these published reports: An NPC the etiology of Gradenigo's syndrome; the presence of recurrent symptoms of the syndrome persisted in a patient. > Case [cancerjournal.net]

Epidemiology

An awareness of its existence and appropriate levels of suspicion of the condition are necessary to prevent severe damage or death in those affected. [ 2, 3 ] Epidemiology There are no reliable recent figures. [patient.info]

Epidemiology Frequency Petrous apicitis was commonly encountered before the introduction of antibiotics. It now appears quite rarely. [emedicine.medscape.com]

壊死性胆嚢炎 necrotizing cholecystitis 壊死性腸炎 necrotizing enteritis(enterocolitis) 壊死性リンパ節炎 necrotizing lymphadenitis 壊疽 gangrene ガス壊疽 gas gangrene 糖尿病性壊疽 diabetic gangrene フルニエール壊疽 Fournier's gangrene 壊疽性口内炎 gangrenous stomatitis 衛星現象 satellite phenomenon 疫学 epidemiology [docsplayer.net]

Pathophysiology

Pathophysiology Caused by extension of an infection of the middle ear, mastoid sinus, or both to the petrous apex that occurs because of extensive pneumatization and the presence of bone marrow. [accesspediatrics.mhmedical.com]

We hope to add new information to the existing literature and to aid in the understanding of the pathophysiology and management of this condition. [tandfonline.com]

Gradenigo classically described the following as the classic features of this syndrome: Discharging ear Retro orbital pain Adbucent nerve paralysis causing diplopia Causative factors: Uncontrolled mastoiditis Epidural abscess following mastoiditis Pathophysiology [drtbalu.co.in]

The potential role of hypocortisolism in the pathophysiology of stress-related bodily disorders. Psychoneuroendocrinology, 25(1), 1-35. ‎ Pagina 52 - In: Wall PD, Melzack R (eds), Textbook of pain. Churchill Livingstone, Edinburgh... ‎ [books.google.it]

Pathophysiology Bacteria travels from the middle ear to the mastoid air cells, which contain highly vascular marrow and are susceptible to infection [5]. [eyewiki.aao.org]

Prevention

There is little evidence to support increased use of antibiotics in acute otitis media to prevent this complication. [ncbi.nlm.nih.gov]

Suspicion of Gradenigo's syndrome (GS) is essential since early treatment is required to prevent permanent sequelae. [elsevier.es]

[…] antibiotics in cases of acute otitis media ( where they are felt to be indicated) may help to prevent chronic presentations due to partially treated mastoiditis. [patient.info]

Early recognition of condition is important to prevent intracranial complications like Meningitis, Intracranial abscess, Spread to skull base and involvement of IX, X, XI cranial nerves (Vernet’s syndrome), Prevertebral/parapharyngeal abscess, Spread [neuroradiologycases.com]

References

  1. Devic M, Boucher M, Raveau M. Some cases of Gradenigo-Lannois syndrome. Journal de médecine de Lyon. 1996;47 (96):537–547.
  2. Bléry M, Chagnon S, Picard A, Babin C. Cranial osteitis: a report on four cases, including a Gradenigo-Lannois syndrome (author's transl). Journal de radiologie. 1980;61(11):677–681.
  3. Motamed M, Kalan A. Gradenigo's syndrome. Postgraduate medical journal.2000;76(899):559-560.
  4. Sherman SC, Buchanan A. Gradenigo syndrome: a case report and review of a rare complication of otitis media. J Emerg Med. 2004;27:253–256.
  5. Valles JM, Fekete R. Gradenigo Syndrome: Unusual Consequence of Otitis Media. Case Rep Neurol. 2014;6(2):197–201.
  6. Ibrahim M, Shah G, Parmar H. Diffusion-weighted MRI identifies petrous apex abscess in Gradenigo syndrome. J Neuroophthalmol. 2010;30:34–36.
  7. Pedroso JL, de Aquino CC, Abrahão A, et al. Gradenigo's Syndrome: Beyond the Classical Triad of Diplopia, Facial Pain and Otorrhea. Case Rep Neurol. 2011;3:45–47.
  8. Yoong HS, Kiaang TK. Gradenigo's syndrome presenting as a tumor. Otolaryngol Head Neck Surg. 2006;135:821–822
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